< Current issue
Dermatopathology: Practical & Conceptual October - December 2003
New Heights: “Hypopigmented mycosis fungoides” is not always mycosis fungoides!
Betina Werner, M.D.
Sonya Brown, M.D.
A. Bernard Ackerman, M.D.
The issue of hypopigmentation in mycosis fungoides
Our method for attempting to assess authenticity of a hypopigmented expression of mycosis fungoides
Definition of mycosis fungoides and criteria morphologically (clinical and histopathologic) for diagnosis of it
“Hypopigmented mycosis fungoides” in historical perspective
Our conclusions about “hypopigmented mycosis fungoides”
Summary of attributes of the patients who surely had “hypopigmented mycosis fungoides”
Photographs of clinical lesions and photomicrographs of the 19 patients with unquestionable hypopigmented mycosis fungoides
Summary of attributes of the patients who did not have “hypopigmented mycosis fungoides”
Photographs of clinical lesions and photomicrographs of the four patients who did not have hypopigmented mycosis fungoides:
Reasons we were unable to make a specific diagnosis of mycosis fungoides in some patients reputed to have it
Conclusions in the form of five questions and our own answers to them
How to differentiate, clinically and histopathologically, pityriasis alba and vitiligo from hypopigmented mycosis fungoides
A patient with stereotypical mycosis fungoides associated with hypopigmentation
A patient with stereotypical mycosis fungoides associated with both hypo- and hyperpigmentation
"Hypopigmented mycosis fungoides" in historical perspective
It was in 1973, just 30 years ago, that "hypopigmented mycosis fungoides" was mentioned for the first time by Ryan
in the context of a proposal by them about "epidermal origin" of mycosis fungoides. Those co-workers suggested that "foci of abnormal cells might begin to proliferate and spread within the epidermis, first provoking an inflammatory response in the dermis and only later colonizing it." That hypothesis, according to them, would explain "the prolonged inflammatory phase in which a banal infiltrate hugs the underside of the epidermis." As evidence in favor of their proposition, Ryan and associates called attention to one patient, among others, with hypopigmented patches in company with "infiltrated papules" of mycosis fungoides. They noted "early involvement of the basal layer of the epidermis" and indicated that it was responsible for "pigmentary changes" that could be seen in the course of the disease, among those changes being hypopigmentation alone and hypopigmentation as a component of poikiloderma. The authors averred that "the early pigmentary changes, the PAS-positive globules above the epidermal basement membrane and the supra-papillary fibrosis could also be explained more easily if mycosis fungoides primarily affects the epidermis rather than the dermis." Although the hypothesis of Ryan
about biological aspects of mycosis fungoides was abstruse, at best, both the clinical picture and the photomicrograph shown by them indicate that the patient likely had mycosis fungoides.
Smith and Samman,
in 1978, were the first to describe, in detail, a patient with "hypopigmented mycosis fungoides." That 14-year-old West Indian boy was said to have developed a "gradual appearance of circular and oval non-itchy hypopigmented patches on the skin of his trunk and limbs over a 10 year period." The authors commented that "in our patient the diagnosis of early mycosis fungoides is based on the histological appearances which are typical of those seen in parapsoriasis lichenoides and parakeratosis variegata." Two clinical pictures and two photomicrographs were shown, all four of them displaying changes consonant with a diagnosis of mycosis fungoides.
In 1982, Breathnach
reported on "ultrastructural observations" about "hypopigmented mycosis fungoides," the patients being five dark-skinned individuals with "widespread, well-demarcated hypopigmented lesions." The disease in one of those patients already had been the subject of a publication by Smith and Samman.
Breathnach and associates showed clinical pictures of "case 1" and "case 2" ("case 1" was the patient reported on previously by Smith and Samman) and one photomicrograph of the patient referred to as "case 2." They described the clinical features of the disease in general, but provided no details about the actual appearance of lesions in either patient, stating only that all patients had non-pruritic hypopigmented macules over the trunk and limbs, and that three patients had some degree of "scaling and infiltration" (case 2 among them). Among the five patients presented, only for "case 2" was information sufficient provided to enable us to attempt to make a diagnosis with precision. On the basis of a clinical picture and a photomicrograph, we were able to conclude that the patient probably did have mycosis fungoides. Ultrastructural studies of the skin affected by mycosis fungoides permitted the coworkers to find "melanocytes within hypopigmented lesions [that] exhibited evidence of degeneration, including marked dilatation of the rough endoplasmic reticulum, swelling of mitochondria with loss of cristae, and cytoplasmic vacuolation" and "abnormalities of melanogenesis, characterized by the production of small spherical incompletely melanized melanosomes." Breathnach and co-workers saw no evidence of "a block in transfer of melanosomes from melanocytes to keratinocytes."
In the same year, 1982, Zackheim
shared their experience with three dark-skinned individuals who had "mycosis fungoides in which all or most of the lesions were hypopigmented." The authors offered details of the lesions clinically, photographs of the gross appearance of the lesions in all three patients, and photomicrographs of sections of tissue from biopsy specimens in patient one and patient three. All of the photomicrographs were "shot" at high power, one of them showing an unquestionable spongiotic dermatitis. Patient two, a 75-year-old woman with a widespread pruritic "eruption," hypopigmented patches, and a lymph node that enlarged in the course of the disease, likely had mycosis fungoides on the basis of features grossly. In the absence of illustrations that captured the essence of findings histopathologically, however, we were unable to come to a diagnosis with specificity in that patient. Because illustrations pertinent to the other two patients were not sufficiently revealing, we could not render a precise diagnosis for them either.
in 1985, told of their experience with a 35-year-old black woman who had "numerous asymptomatic vitiligo-like small hypopigmented macules varying from 2 to 10mm in diameter on the upper and lower extremities." No other lesions indicative of mycosis fungoides were mentioned and no follow up of the patient was provided. Moreover, in one of the photomicrographs, the findings are more in keeping with those of a lichenoid dermatitis than of mycosis fungoides. Nonetheless, the other photomicrograph surely exhibits some changes reminiscent of those in "pagetoid reticulosis." Despite that, it is unlikely that the patient truly had mycosis fungoides.
in 1986, detailed the results of their studies of the "immunopathology of hypopigmented mycosis fungoides" in two patients said by them to have that disorder. The collaborators were interested particularly in the immunohistochemical expression of lymphocytes in mycosis fungoides. A photograph of clinical lesions and a photomicrograph of one of the patients were shown, a 35-year-old Venezuelan man who had a longstanding history of generalized pruritus and an "increasing number of hypopigmented and hairless areas on the trunk, buttocks and backs of the legs." Physical examination revealed the following: "poorly defined areas of hypopigmentation on the inner surfaces of both upper arms and multiple well-defined circular and oval hypopigmented hairless macular lesions on the buttocks and upper thighs. Some of the lesions were dry, scaly and erythematous and others contained prominent erythematous follicles." In the photomicrograph can be appreciated slight hyperkeratosis, slight epidermal hyperplasia with scattered lymphocytes arranged in small clusters and as solitary units in the basal layer or above it. Lymphocytes are evident around venules and in the interstitium of the reticular dermis where they repose between thickened bundles of collagen, but also in dermal papillae. In brief, one of the patients of Rustin
almost certainly had mycosis fungoides. Regarding immunohistochemical aspects, the authors found that CD8+ (cytotoxic/supressor) T lymphocytes predominated in dermal infiltrates and in the epidermis of sections from both biopsy specimens, in contrast to what they claimed was the usual situation in mycosis fungoides, namely, preponderance of CD4+ (helper) T lymphocytes.
In 1986, Goldberg
speculated about the mechanism of "hypopigmentation of mycosis fungoides" predicated on findings in a 27-year-old Puerto Rican woman who presented herself with a "10-year history of pruritic patches on her neck and shoulder with progressive loss of pigmentation from her face and arms, and presence of a poikilodermatous patch in the shoulder." Clinical photographs and photomicrographs were available for perusal. Although clinically a diagnosis of mycosis fungoides cannot be made with confidence (rarely does mycosis fungoides involve the face and in linear fashion), the findings histopathologically are those of mycosis fungoides. In an effort to explain the mechanism of hypopigmentation by way of findings by electron microscopy, Goldberg and co-workers told of having noted "decreased numbers of melanosomes within keratocytes"; "melanocytic melanosomes, however, were numerically and morphologically normal." They concluded that "these findings imply a defect in transfer of melanosomes from melanocytes to keratocytes."
The second example published of "hypopigmented mycosis fungoides" that developed in a child was by Misch
in 1987 in a then 19-year-old West Indian woman with "at least 7-years history of mildly itchy pale patches of skin of the left arm and buttock." Unfortunately, it was not possible for us to assess accurately the nature of the disease in that patient because the authors provided only one clinical photograph, close up, and no photomicrographs of sections stained by hematoxylin and eosin (only immunoperoxidase stains were pictured).
Sigal and co-workers
recounted their experience with a 64-year-old woman who for eight months suffered from maddening pruritus and was noted to have these changes in the skin: "erythematous papules, confluent in areas over the scalp, upper part of the chest, abdomen and thighs; and hypopigmented round or oval areas, poorly defined on the face, surrounded by a papular, erythematous border on the trunk and arms. Some of these hypopigmented lesions were slightly scaly." Although a biopsy of a skin lesion was performed, no photomicrograph is accompanying. The findings in sections of tissue, however, were recorded by them in these words: "massive exocytosis of mycosis cells and Pautrier's microabscesses within the epidermis, but a scanty infiltrate in the dermis." At the time of the biopsy, the patient had an enlarged axillary lymph node. Six months later she developed numerous tumors over the entire integument. After treatments of various kinds, including chemotherapy, the patient died of septicemia and aplasia of the bone marrow. On the basis of the clinical features, the description of findings by conventional microscopy, and biologic course, sequentially, we are compelled to concur that the patient did indeed have mycosis fungoides. Sigal
also studied the lesions ultrastructurally and imunohistochemically. Regarding the first matter they reported that "most melanocytes underwent disintegration; in the others, swelling of mitochondria with loss of cristae was observed; melanosomes were almost spherical and incompletely melanized; melanin containing macrophages were present in the upper dermis." Study by direct immunofluorescence revealed that "all the lymphocytes within the epidermis and dermis stained with Leu 1 (CD5) and OKT4 (CD4)."
Cooper and colleagues,
in 1992, reported on a 68-year-old black woman with a history of "increasing numbers of asymptomatic white spots on her legs and arms." On physical examination, she was observed to have "numerous hypopigmented macules on her thighs, upper extremities and abdomen," but "erythema, scaling, or atrophy was not appreciable in any of the lesions." A photomicrograph shows a normal cornified layer, a spinous zone that harbors several lymphocytes in company with slight spongiosis, and an infiltrate of lymphocytes around superficial venules and in lichenoid array. The written and pictorial information provided do not enable us to conclude that the patient did, in fact, have mycosis fungoides. Clinically, all the lesions were small and devoid of erythema or scale; none were patches. The findings histopathologically lack specificity; they could represent a true spongiotic dermatitis or the inflammatory phase of vitiligo. In short, we are not convinced by the evidence presented that this patient had mycosis fungoides.
also in 1992, shared findings in a 25-year-old Jamaican woman with "multiple circular and oval lesions on the trunk and limbs showing varying degrees of hypopigmentation." The images, clinical and histopathologic, presented by the authors force us to agree that the patient truly had mycosis fungoides.
Volkendandt and colleagues,
in 1993, wrote about the "molecular detection of clone-specific DNA in hypopigmented lesions of a patient with early evolving mycosis fungoides." Their patient was a 32-year-old man with "multiple hypopigmented, partially confluent skin lesions on his trunk and arms" and "some areas were characterized by reddish plaques with slight scaling" and, two years later, "reddish plaques and nodules." The photographs of clinical lesions and the photomicrographs allowed an inference to be drawn by us that the patient did have mycosis fungoides. Regarding the molecular detection of clone-specific DNA, the authors utilized the polymerase chain reaction employing a rearranged sequence of a T-cell receptor to demonstrate clonality of the lymphocytes.
In 1994, Ratnam and Pang
published a "clinico-pathologic study and five-year follow-up of 10 cases of hypopigmented mycosis fungoides." They studied patients whose lesions fulfilled the following criteria "(i) hypopigmented macular patches, not due to vitiligo; no preceding history of inflammation; no loss of sensation and negative findings of Malassezia furfur on skin scraping" and "(ii) a histological picture consistent with mycosis fungoides consisting of lymphocytic epidermotropism with or without Pautrier's microabscesses." The authors showed a photograph of clinical lesions before and after treatment with PUVA of just one of the 10 patients. Moreover, the data pertinent clinically to the 10 patients were presented in a table, but no details were supplied for each patient. No photomicrographs were shown. In short, it was impossible for us to assess comprehensively the disease(s) in patients presented by Ratnam and Pang in regard to authenticity of the diagnosis of mycosis fungoides.
In 1994, Whitmore
presented that which they learned about "three African-American males, ages 9, 15 and 22 years, that [sic] were found to have mycosis fungoides on evaluation of skin biopsy specimens after initially being diagnosed with, and treated for, pityriasis alba." They pictured clinical lesions and showed photomicrographs of sections from specimens of two of the patients, the 9-year-old and the 15-year-old. Both patients had asymptomatic hypopigmented patches on the face, a presentation somewhat unusual for mycosis fungoides. The photomicrographs did not convey changes diagnostic of mycosis fungoides. We find it impossible to determine whether the three patients reported on by Whitmore
really had mycosis fungoides.
In 1995, the subject of "hypopigmented mycosis fungoides" was addressed by Robert and colleagues
Annales de Dermatologie et de Venereologie
. They reported on a 28-year-old woman from Mali thus: "[she] developed non-infiltrated hypopigmented and discretely squamous macules involving the entire body. The diagnosis of mycosis fungoides was made on pathology evidence of epidermotropic lymphocyte infiltration with a few small nests." The authors depicted two lesions clinically and one section of tissue that was too dark for any detail to be appreciated histopathologically. Based on the illustrations presented, a diagnosis of mycosis fungoides cannot be made by us with surety.
Lambroza and co-workers,
also in 1995, gave an account of seven patients with "hypopigmented mycosis fungoides." To illustrate "cases" one and two, the authors showed photographs both of clinical lesions and histopathologic findings, thereby enabling us to attempt clinico-pathological correlation. Case one was a 21-year-old black man with "asymptomatic, hypopigmented eruption of two years duration on the trunk, buttocks, and extremities" and "8-10 mm macules, covered with a fine scale, especially prominent on the extensor surfaces of the extremities and buttocks." Case two was a 29-year-old black woman with a "4-year history of progressive, generalized depigmentation that began on her right elbow." She, too, had "diffuse loss of pigment on her forearms, lower extremities, and trunk without atrophy or telangiectasia." Photomicrographs showed similar changes in sections from both biopsy specimens, to wit, slight to moderate orthokeratosis, lymphocytes in small clusters within the epidermis or disposed as solitary units along the dermo-epidermal junction, and thickened bundles of collagen in association with an interstitial infiltrate of lymphocytes. The features clinically, coupled with the changes histopathologically, permitted us to conclude that at least two of the series of seven patients with "hypopigmented mycosis fungoides" actually had that lymphoma. About the others, there simply was not enough information given to allow a diagnosis to be made by us with certainty.
El-Hoshy and Hashimoto,
in 1995, too, presented a patient with "hypopigmented mycosis fungoides," that being a 16-year-old black man who, for 12 months, had an asymptomatic rash on the upper extremities and thighs. Examination clinically revealed "four hypopigmented plaques and macules on both upper and lower extremities" and "two erythematous nodules measuring 1 cm in diameter were seen, one each on the plaque of the left upper arm and right forearm." The authors captured pictorially the lesions on his thigh and a section of tissue from a biopsy specimen taken of one of the lesions. Scrutinity of the photographs and photomicrographs permit the inference that the patient did, in fact, have mycosis fungoides.
In 1996, Amichai
wrote about "hypopigmented mycosis fungoides in a white female." They reported on a 32-year-old woman who "suffered from the intermittent appearance of indolent hypopigmented areas during the previous five years." On physical examination the co-workers found "several well-demarcated, hypopigmented macules up to 2 cm in diameter present on her body and limbs." Assessment of the photograph of clinical lesions and the photomicrograph, even though the latter does show some attributes reminiscent of those of mycosis fungoides, does not enable us to conclude that the patient had mycosis fungoides. The presentation clinically is too subtle, all the lesions being asymptomatic, and all devoid of scales. Although we cannot make a diagnosis with exactness, we favor vitiligo.
in 1997, reported on 24 patients with mycosis fungoides, in each of whom the disease began before age 20 years. Six of the patients presented themselves with hypopigmented macules. The only pictures provided (both photographs of clinical lesions and photomicrographs) were from one patient with a peculiar widespread "chronic scaly dermatitis" that started at six months of age and, in the course of the succeeding several years, evolved to "psoriasiform scaly plaques" and hypopigmented macules. At age five, a clinical diagnosis of "guttate parapsoriasis" was rendered. According to the authors, at age 17 the patient started to show features of "poikiloderma" and in adulthood the condition never improved, pruritus and dryness being experienced especially on the buttocks and posterior aspect of the upper part of thighs. Zackeim
pictured the patient clinically at age 25 and they showed a photomicrograph of tissue from a biopsy specimen taken from the patient at age 22 months and another at age 25 years. Despite the onset of the disease early in life, the findings in sections from the first biopsy specimen are those of mycosis fungoides. Nothing of consequence can be concluded about the other 23 patients in this series because too little information about them was provided.
In the same year, 1997, Di Leandro and co-workers
wrote of "a case of hypopigmented mycosis fungoides in a young Caucasian boy." According to them, the youngster had "hypopigmented macular patches [sic] on his right flank, manifesting at age 7 years and never regressing." Physical examination revealed "multiple circular to oval macules and patches on the right side, with extension on the pubic and gluteal regions" and "only the largest had in its center mild erythema with fine scale." Although the article is illustrated richly with three pictures of clinical lesions and two photomicrographs, the quality of the latter is poor. Nevertheless, on the basis of all the information presented, we are confident that the patient had mycosis fungoides.
Robert and colleagues
wrote a letter in 1998 to the editor of the
British Journal of Dermatology
in which they told of a 32-year-old white woman who had "an asymptomatic hypopigmented macular eruption on both thighs and the left calf of one year's duration that clinically resembled vitiligo or pityriasis alba." According to the authors, "the macules were barely visible on examination" and were "non-scaling, non-infiltrated hypopigmented macules, around 4 cm in diameter." In addition to a photograph of the lesions clinically, Robert
showed a photomicrograph "shot" at high power in which the stratum corneum was seen to be normal, several lymphocytes were present along the dermal-epidermal junction and in the spinous zone in association with scant spongiosis, and a somewhat lichenoid infiltrate of lymphocytes was apparent in company with slightly thickened collagen bundles. The clinical features and histopathological findings simply are not sufficient for us to make a diagnosis with specificity of mycosis fungoides.
In the following year, 1999, Grunwald and Amichai
told of a 12-year-old Caucasian young man who presented himself with a "single hypopigmented lesion of mycosis fungoides." The patient was said to have "an asymptomatic lesion on his left thigh of five years duration." On physical examination, "a single, slightly scaly, hypopigmented plaque, 5 cm in diameter, was seen is his left thigh." Grunwald and Amichai showed a photograph of the clinical lesion and a photomicrograph which was "shot" at high power magnification, but which fails to depict signs of indubitable mycosis fungoides. In short, neither the clinical features nor the histopathological findings allow for diagnosis of mycosis fungoides.
in 1999, reported on findings in a 7-year-old black boy who presented himself with a "3-year history of slowly developing, hypopigmented patches scattered over his buttocks, legs and arms involving approximately 20% of his body surface." The authors focused mainly on virologic aspects because the boy was negative serologically for HTLV-I/II (human T-cell lymphotrophic virus), which was somewhat unusual for an individual coming from a region (Grenada) endemic for that virus. His lymphocytes, however, harbored the "tax" proviral sequence. The authors came to this conclusion: "the present case strengthens the concept that HTLV-I or a very closely related virus is associated with mycosis fungoides." Apart from the debate about viral cause for mycosis fungoides, which is not relevant to this Arbeit by us, the photograph of clinical lesions and the photomicrograph presented by Zucker-Franklin
convey that the patient did have mycosis fungoides.
Quaglino and associates,
in 1999, published a communication concerning seven patients who were diagnosed as having mycosis fungoides before they had reached 20 years of age. The co-workers analyzed the course of the disease in those patients and reviewed the medical literature on the subject of mycosis fungoides that presented itself clinically early in life. Regarding hypopigmentation, a 12-year-old girl had "hypopigmented macules on her left thigh, covered with a fine scale." There was a photograph of the lesions clinically in that patient, but there was no photomicrograph and no description of the findings histopathologically. We were not able to judge the authenticity of the diagnosis of mycosis fungoides based solely on the clinical features pictured. No mention was made by Quaglino
of hypopigmentation in the other six patients.
In 2000, Choe
wrote about features clinically and findings histopathologically in a 28-year-old woman who "initially presented with an asymptomatic hypopigmented skin eruption of seven years duration." Examination revealed "whitish 1-5 cm sized macules and patches on her buttock, lower abdomen, upper arms, knees, and elbow." A photograph shows clinical lesions in the axilla and the photomicrograph portrays a basket-woven pattern of the cornified layer, scattered lymphocytes at all levels of the viable epidermis, some of those mononuclear cells being disposed as solitary units and others in small collections, absence of spongiosis, and a dense infiltrate of lymphocytes in the upper part of the dermis. On the basis of the visual material presented by Choe
, we are given to agree with them that the patient had mycosis fungoides.
Also in 2000, Bouloc and co-workers
studied "leucoderma" in four patients with "erythrodermic cutaneous T-cell lymphomas (two with erythrodermic mycosis fungoides and two with Sézary syndrome)." The authors seem to imply that Sézary syndrome is not a manifestation of mycosis fungoides, a concept with which we do not concur. We do agree with Bouloc and co-workers, however, when they affirm that "these cases must be distinguished from previously described hypopigmented mycosis fungoides where hypopigmented lesions were the sole manifestation of the lymphoma." All four patients were 63 years old or older at the time the vitiligo-like changes appeared, and in all instances the depigmentation followed an erythroderma of longstanding. Because hypopigmentation was not the most striking or the most important feature clinically at the time the patients presented themselves, we have not included those four patients in our analysis; they do not have "hypopigmented mycosis fungoides" as it is understood generally to be.
in 2000, recorded their observations of nine patients in Singapore with "mycosis fungoides diagnosed before the age of 21 years"; "eight of the nine patients presented with hypopigmented patches and plaques." The colleagues showed pictures of hypopigmented patches in two patients. Photomicrographs were not provided of sections of specimens from any patient. According to the authors, the diagnosis of mycosis fungoides by conventional microscopy was established when the following criteria were met: "diffuse infiltrate of a large number of cells with highly irregular, darkly stained nuclei (mycosis cells) in the upper dermis" and "this infiltrate invades the epidermis in some areas and may form Pautrier's microabscesses." We were not able to judge the accuracy of the diagnosis of mycosis fungoides in any of the eight patients presented by Tan
because of paucity of information conveyed by the illustrations. Furthermore, the criteria applied by them for the diagnosis histopathologically of mycosis fungoides are not in synchrony with our own and, in our estimation, simply are not decisive. Although "highly irregular, darkly stained nuclei (mycosis cells) in the upper dermis" and "Pautrier's microabscesses" may be encountered episodically in patches of mycosis fungoides, they are present only exceptionally. In brief, neither nuclear atypia of lymphocytes nor collections of them in the epidermis are expected findings in patches of mycosis fungoides.
Akaraphanth and associates,
in 2000, reported on the follow-up of nine patients with "hypopigmented mycosis fungoides." They stated that "the diagnosis was established by the appearance of clinical lesions (hypopigmented macules, patches, and thin plaques) and confirmed histologically." A table with a summary of clinical information on each patient and photographs of clinical lesions of two patients was provided. Neither photomicrographs nor detailed description of findings histopathologically were presented. It was impossible, therefore, for us to decide whether any of the patients followed by Akaraphanth
had mycosis fungoides.
In 2000, too, Neuhaus and collaborators
told of their experience with "purely hypopigmented mycosis fungoides of 8-years duration in a 18-year-old woman who responded readily with PUVA treatment." Physical examination of the patient revealed "hypopigmented, nonscaly, irregularly bordered macules and patches on her left flank, abdomen, left thigh, and right arm involving less than 10% of the skin." According to the co-workers, assessment of sections from the biopsy specimen disclosed the following: "prominent epidermotropism by large, atypical lymphocytes, either single or grouped, with extension along the dermoepidermal junction. A mild, superficial, dermal, perivascular, lymphoid infiltrate was present, with extension and alignment of atypical lymphocytes between wiry collagen bundles." The authors pictured a single photograph of the clinical lesions and one photomicrograph, those two illustrations allowing a conclusion to be reached that the patient had mycosis fungoides.
also in 2000, presented five patients with "hypopigmented mycosis fungoides" and concentrated on the presence or absence of clonality in lymphocytes involved in the process. They performed analysis of T-cell receptor gene rearrangement in specimens of skin from four patients, a positive result being obtained in three of them. In every patient, the authors commented specifically on clinical features and histopathologic findings. But only in one patient (# 3) were both a photograph of clinical lesions and a photomicrograph provided. In two patients, only a photomicrograph was shown; in another, only the clinical lesions were pictured, and in the last patient, no pictures were shown at all. Patient 3 was a 43-year-old African-American woman with an "asymptomatic skin eruption of 2 years duration" and "multiple hypopigmented slightly scaly patches, involving the extremities and trunk." The photomicrograph "shot" at high magnification is so dark that all details are obscured. In sum, none of the five patients presented by Qari
could be recognized by us as having mycosis fungoides because too little pictorial was provided.
in 2000, published their findings in seven patients with "unilesional mycosis fungoides," among them being a 14-year-old boy with a "slightly scaly, hypopigmented patch from the age of 7, diagnosed as either nevus anemicus or unusual pityriasis alba, without histological verification." There was a picture of the clinical lesion of that patient, but no photomicrograph. The changes histopathologically were described in these words: "In all the patients the biopsy specimens revealed hematoxylin-eosin findings diagnostic of early mycosis fungoides, including various degrees of dermal lymphocytic infiltrate with significant epidermotropism of solitary haloed lymphocytes exhibiting nuclear atypia." In the absence of a single illustration of a section of tissue, we are unable to either agree or disagree with the diagnosis in that patient of mycosis fungoides.
In the following year, 2001, Stone and co-workers
studied, retrospectively, seven patients with "hypopigmented mycosis fungoides." They gave a detailed description of clinical features and histopathologic findings in two of those patients and published photographs of clinical lesions and photomicrographs in both of them. Patient 1 was a 36-year-old black man with "more than 10-year history of slightly pruritic, hypopigmented macules on his arms, legs and trunk, involving approximately 40% of his total body surface"; "the lesions were poorly circumscribed and a few were slightly atrophic." Patient 2 was a 56-year-old woman with "pruritic, slightly scaly hypo- and hyperpigmented macules, patches, and plaques on her arms, legs, trunk, face, scalp and buttocks." She claimed to have "first noticed 'ringwormlike' skin lesions on her anterior upper chest approximately 34 years previously" and "seven years prior to presentation, the lesions began to spread and become pruritic." Stone
stated that two years after the initial consultation, the patient presented herself with a "2 to 3-month history of generalized erythematous plaques, nodules, and tumors in the previous areas of hypopigmentation," as well as "otitis media, thrush and severe infection with genital herpes simplex virus." There was fatal progression to "acute distress syndrome," at which time the patient was found, by examination radiographically, to have "generalized lymphadenopathy and bilateral infiltrates." All features clinically and findings histopathologically of patients 1 and 2 are consonant with mycosis fungoides. In the remaining five patients it is not possible to affirm that diagnosis because the authors failed to offer sufficient data to enable clinico-pathological correlation.
In 2002, Shabrawi-Caelen
shared their thoughts about the "clinical, histopathologic, immunohistochemical, and molecular characteristics of hypopigmented mycosis fungoides in 15 patients." Of the 15 patients with hypopigmented mycosis fungoides, the authors pictured three clinically, namely, patients number 1, number 7 and number 9. They did not make clear, however, whether the photomicrographs shown in figures "2A" through "2E" are of sections from a single patient or from more than one patient, and, moreover, whether the sections came from a biopsy specimen taken from any of the three patients illustrated by them clinically, to wit, numbers 1, 7 and 9. That failure prevents us from coming to a conclusion with confidence about how many of the 15 patients of Shabrawi-Caelen
actually had mycosis fungoides. The features pictured clinically in the three patients are consistent with those of mycosis fungoides and the findings shown histopathologically also are those of mycosis fungoides. What is lacking, though, is any indication by the authors of which, if any, of the three patients pictured had the changes shown in the photomicrographs. In regard to immunohistochemical studies that were performed, the authors commented that "in most of the specimens we examined, the epidermotropic lymphocytes were predominantly CD8+, indicating a T-supressor lineage, unlike the mature CD4+ T-helper cell phenotype that is usually encountered in mycosis fungoides."
in 2003, reported on seven Caucasian patients with hypopigmented mycosis fungoides, two of whom were children and five were adults. The authors pictured three patients clinically (4, 5, and 7) and one photomicrograph of a section from a specimen of skin taken from patient 5. Clinico-pathologic correlation, therefore, was possible only in patient 5, a 32-year old male who, at age 16 in 1988, had "multiple, confluent hypopigmented lesions on trunk and arms associated with small areas of erythema." In 2002, he presented himself with "hypopigmented lesions associated with erythematous patches, plaques, and small tumors on the left flank." The findings in the photomicrograph are consonant with mycosis fungoides. Ardigo
also performed, in all patients, T-cell receptor gene rearrangement by the technique of polymerase chain reaction, as well as molecular studies after laser-beam microdissection of the epidermis in five of the patients. A T helper phenotype was found in 57% of patients and monoclonality of the lymphocytic infiltrate was detected in all of them.
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