The Olmsted syndrome

A 35-year-old man had a hyperkeratotic disorder of the palmoplantar skin since the age of 6 months. The palmoplantar keratoderma progressed to thick and warty hyperkeratotic plaques, which enlarged and formed verrucous lesions and deep fissures. The acral keratoderma gradually involved the dorsal surface of the hands and feet with flexion contractures of the fingers and toes (Fig. 1). Since the age of 2 years, the patient also showed universal alopecia and small sharply marginated hyperkeratotic plaques around the nose and mouth, in the groin, and in the intergluteal area (Fig. 2). All of these keratotic lesions were strikingly symmetric.
Examination of the oral mucosa revealed a white plaque over the left lateral border of the tongue; crusted white verrucous plaques were found at the labial commissures and underneath the lower lip. There was complete absence of the nails and the roentgenographic examination of the hand showed pronounced osteolysis of the distal phalanx of each of the fingers. The palmoplantar keratoderma has been complicated by the development of constricting bands of keratin around the bases of the fingers, leading to their strangulation and autoamputation (Fig. 1). The mental and physical appearance were normal. A family history of similar verrucous lesions was lacking. Histopathologic examination of the palmar skin showed massive parakeratotic hyperkeratosis and considerable acanthosis. In the thickened horny layer, many cells showed perinuclear edema. The patient was treated with topical retinoic acid cream, 0.1%, at night. This produced an improvement in the plaques around the nose and mouth.     

Annular atrophic plaques of the skin (Christianson's disease)

A 69-year-old Caucasian man presented to the Gainesville Veterans Administration Medical Center for evaluation of several asymptomatic enlarging lesions on the face and forearms that had been present for 10 to 15 years. They were initially small but had progressively enlarged, especially during the previous 5 years. He reported having sustained a concussive grenade blast injury of the left temple and right forearm during the Korean Conflict in 1951. The injured areas healed uneventfully in 2–3 weeks. The patient was otherwise healthy, and the review of systems was noncontributory. Laboratory work-up (complete blood count, chemical profile, erythrocyte sedimentation rate, rheumatoid factor, hepatitis profile, antinuclear antibodies, urinalysis, and chest X-ray) was within normal limits. Lyme antibody titers were negative. Physical examination revealed similar-appearing lesions located variously on both temples, left preauricular and infraauricular areas, and the right forearm (Fig. 1). The lesions were large (ranging from 4 to 12 cm in diameter), centrally atrophic patches with ivory-colored thickened edges, and had the distinct appearance of healed skin grafts. Blood vessels were easily seen through the atrophic skin. All lesions were located on sun-exposed areas (predominantly on the patient's left side). No similar lesions elsewhere on the body or skin graft donor sites were found. The patient was treated with doxycycline 100 mg by mouth twice a day for 21 days to no avail, Intralesional steroids and clobetasol propionate ointment under occlusion were used without any change in the lesions. At the submission of this paper, the lesions remain stable. Skin biopsies were taken from the edge and center of the lesions, Histologic examination (Fig. 2) of the center of a lesion showed a flattened epidermis without interface changes. Sclerosis of superficial and mid, but not deep, dermal collagen was present, particularly within the specialized connective tissue surrounding eccrine structures. A focus of lymphocytic inflammation with rare plasma cells and edema was present at the dermal-subcutaneous interface. Histologic examination of the edge of the lesion showed a flattened epidermis overlying a perivascular and periadnexal lymphohistiocytic inflammatory infiltrate. The papillary dermis was sclerotic centrally, and sclerosis was present around one eccrine unit. Increased interstitial mucin was noted. Direct immunofluorescence using antibodies to IgG, IgM, IgA, and C3 was negative, A silver stain for borrelia organisms was negative. Electron microscopy of a biopsy from the advancing border of a lesion revealed foci of complete cytoiysis of the basilar epidermis above an interrupted basal lamina (Fig, 3a). Cellular debris was evident even within the subajacent papillary dermis (Fig. 3b). Abundant colloid-like material associated with occasional 8–10-nm straight tubules was also present within the upper papillary dermis. A solitary lymphocyte lay closely apposed to a basilar keratinocyte having large cytoplasmic vacuoles. Electron microscopy of a biopsy from the center of the same lesion revealed a flattened basilar epidermis and focal reduplication and interruption of the basal lamina (Fig. 3c). Abundantly interspersed among the collagen bundles of the upper and lower papillary dermis were aggregates that appeared amorphous at low magnification. Upon higher magnification, however, the aggregates could be seen to be comprised of straight and wavy tubules embedded in a finely granular matrix (Fig. 3d). A dermal blood vessel was surrounded by several basal laminae, just outside of which were presumptive lymphocytes with cerebritorm nuclei and histiocytes. No dermal elastic fibers could be identified. Collagen fibrils appeared normal.     

Acrokeratoelastoidosis

A 45-year-old white woman presented with several years' history of firm, shiny papules on the lateral hands with slight extension to the dorsal fingers. The lesions first appeared between the index fingers and thumbs on both hands. They gradually increased in number, coalescing into plaques and affecting the junction between the palmar and dorsal skin. The patient did not have involvement of her feet. She had been diagnosed previously with chronic eczema that had failed to respond to multiple topical medications. In addition, the patient's sister had similar lesions on both hands. The patient denied any symptoms of hyperhidrosis, excessive sun exposure, or trauma. The plaques were asymptomatic, but were cosmetically unappealing to the patient. On physical examination, small, firm, skin-colored, hyperkeratotic papules, coalescing into plaques, were located on the junction between the palmar and dorsal skin on both lateral margins of the thumb and on the radial side of the index finger (Fig. 1). There were no lesions on the feet. A biopsy taken from a papule on the patient's left hand was consistent histologically with acrokeratoelastoidosis. The biopsy showed marked degeneration of collagen in the dermis with solar elastosis and some smudging of the papillary dermal collagen (Fig. 2). She was treated with clobetasone cream to the affected areas on the hands. After 6 weeks of treatment, she reported no significant improvement.     

Lipoid proteinosis: report of a possible localized form on both hands and wrists

A 28-year-old woman was seen for pruritic lesions on both hands and wrists which had been present since the age of 10 years. Both palms showed symmetric, diffuse hyperkeratosis extending over both wrists and the dorsal aspects of both hands with well-demarcated, erythematous, lichenified plaques (Figs 1 and 2). The borders of the plaques were pigmented and studded with papules (Fig. 2). The skin lesions were not related to sun exposure. No similar lesions were found elsewhere on the body. The hair, nails, and dental development were normal. A skin biopsy was taken for histopathologic and ultrastructural studies under the clinical impression of pityriasis rubra pilaris. Microscopically, homogeneous perivascular deposits of hyaline-like material were seen in the papillary and reticular dermis, accompanied by mononuclear cells (Figs 3 and 4). The overlying epidermis was acanthotic with hyperkeratosis (Fig. 3). The hyaline deposits were positive for periodic acid-Schiff (PAS) and oil-red O stains (Figs 5 and 6). Congo red stain was negative. Electron microscopic examination showed abundant amorphous material intermingled with short collagen fibrils in the dermis and around the blood vessels (Figs 7 and 8). Further examination revealed that the patient could protrude her tongue freely. The oral mucosa and vocal cords were smooth. There was no sign of systemic amyloidosis. The patient's complete blood cell count, biochemical tests, and protein electrophoresis were all within normal limits. Antinuclear antibody was negative. Results of porphyrin screening tests were negative for uroporphyrins and coproporphyrins in the urine and feces. The possibility of porphyria was therefore excluded. Both neurologic and ophthalmic examinations were negative. No intracranial calcification was found on a skull X-ray examination. The patient was treated with topical steroid and urea. She was lost to follow-up.     

Vitiligo following the resolution of psoriatic plaques during PUVA therapy

Vitiligo Is an acquired idiopathic disorder, involving 1–4% of tiie world population and characterized by depigmented white patches of the skin that lack the dopa-positive melanocytes.It has been associated with physicai trauma and, systemic and cutaneous diseases. Among the many dermatoses, psoriasis has been reported to be associated with vitiiigo in the same individuals independently, or vitiligo may precede the formation of psoriasis at the same location. Currently, psoralen plus ultraviolet A iight (PUVA) is one of the efficacious treatments of psoriasis and vitiligo with side-effects of hypopigmentation and vitiligo-like lesions. We describe a patient with psoriasis vuigaris in whom vitiligo appeared in the same areas and configurations as his psoriatic plaques as they resolved while being treated with topicai PUVA. A 19-year-old Caucasian man was referred for treatment of his psoriatic flare. His medical history revealed a vitiiigo patch on his right calf at age five. At age 14, he developed initially psoriatic plaques on his knees and elbows which then gradually spread to the legs, arms, hands, trunk, scalp, and the genitai area. The only therapy used was a mid-potency topical steroid ointment with some reiief. Recently the condition had deteriorated enough to seek medical attention. Physical examination revealed sharply demarcated erythematous, silvery-white scaly papules, patches and plaques of various sizes on trunk, extremities, palms, buttocks, and penis. The patient received topical PUVA with 0.1% trimethoxalen cream for 3 months. Uitravioiet A light (UVA) was applied at 0.1 J/cm with an increment of 0.1 J/cm at each session as tolerated. At approximately the tenth session, depigmented lesions were noted around the margins of the regressing psoriatic plaques. This progressed continually until the vitiligous-like lesions completely replaced the resolved psoriatic piaques in exactiy the same configurations (Fig. 1). Skin biopsies of the depigmented areas revealed parakeratosis, hypogranulosis, acanthosis, and a sparse iymphocytic infiitrate around dilated tortuous capillaries in the upper corium (Fig. 2). Also a Fontana-Masson stain showed an absence of melanocytes as may be seen in vitiligo (Fig. 3).     

Cornifying Darier's disease

A 48-year-old Caucasian man recounted the onset of keratotic papules on the trunk at the age of 8 years, with subsequent spread to the forearms, scalp, and forehead. His most severe disease was present on the legs. He complained of pain, itching, and noted exacerbations in the summer and with sweating. The family history was negative. On physical examination, the most striking finding was that of extensive, markedly hyperkeratotic plaques on the lower legs >(Fig. 1). His scalp, forehead, chest, and back exhibited mild involvement, with scattered brown keratotic papules, while his forearms showed mildly hyperkeratotic plaques. Flat-topped brown papules were present on the dorsum of the hands, with a few keratotic papules on the palms, and a few nails with distal notching and red longitudinal streaks. There were no palmar pits or oral mucosal lesions. A shave biopsy was performed of a plaque on the leg, and showed a papillomatous and markedly hyperkeratotic lesion >(Fig. 2). Suprabasal acantholysis in the elongated rete produced characteristic lacunae. The acantholysis was associated with dyskeratosis including corps ronds and grains >(Fig. 3). Together, these features were characteristic of Darier's disease. Treatment years earlier with topical retinoids, topical steroids, topical keratolytics, and multiple oral antimicrobials had been unsuccessful, and isotretinoin had been discontinued due to elevated triglycerides. Treatment was initiated with acitretin and, after 3 months, mild improvement was noted     

Axillary intertriginous granular parakeratosis responsive to topical calcipotriene and ammonium lactate

A 70‐year‐old white man presented to our clinic with a 6–8‐month history of a pruritic, occasionally burning eruption in both axillae. He had been using the same deodorant for more than 1 year and denied any changes in laundry detergent, soaps, or shampoos. He also denied application of other topical products. On physical examination, there were slightly erythematous, lichenified plaques in both axillae, with more extensive involvement of the left side ( Fig. 1 ). Histologic examination of a punch biopsy specimen from a left axillary plaque revealed hyperkeratosis with retention of nuclei and keratohyaline granules in the stratum corneum ( Fig. 2 ). The stratum granulosum was slightly thickened, and the epidermis was mildly acanthotic. Patch tests of the patient's deodorant and shampoo were negative. The patient was advised to discontinue use of his deodorant. His right axilla was treated with topical calcipotriene, applied twice daily, and his left axilla was treated with topical 12% ammonium lactate, applied twice daily. One month later, the lesions in the right axilla had completely resolved. The left axilla was slightly improved, but still exhibited dusky erythematous plaques. After one additional month of treatment with ammonium lactate, the left axillary lesions completely resolved. A follow‐up examination 9 months later revealed no recurrence of the lesions in either axilla.

Figure 1 Open in figure viewer PowerPoint Erythematous, lichenified plaques in the axilla     

An unusual presentation of human papillomavirus (HPV) infection in a black epidermodysplasia verruciformis (EV) patient

A 25-year-old black man had a 20-year history of disseminated plaques on his body. Two years before consultation he noticed several tumors on the genitalia. Physical examination revealed generalized, coalescing, hypopigmented plaques with a very defined and keratotic border that resembled actinic porokeratosis. (Fig. 1). Multiple verrucous and moist tumors were observed on the scrotum (Fig. 2). His past medical history was unremarkable, and no family member had the disease. Histology of the body lesions revealed hyperkeratosis with a horny-layer, basket-weave appearance, large and clear blue-gray keratinocytes with finely granular cytoplasm and vacuolized nuclei, and abundant keratohyaline granules located in the upper epidermal layer (Fig. 3). Scrotum biopsy showed hyperkeratosis, irregular acanthosis with papillomatosis, and koilocytic figures. The genital lesions were treated with monthly cryotherapy plus surgical excision with complete disappearance of the lesions. A decrease in ultraviolet exposure and daily sun-block were encouraged for epidermodysplasia verruciformis lesions. After genital wart clearance, follow-ups are being scheduled every 4 months, with no new lesions to date.     

Disseminated granuloma faciale

A 40-year-old man presented in January 2001 with multiple purple plaques and nodules, which had been present on the back for approximately 3 years. The lesions had gradually extended over the face, trunk and proximal extremities. He had no symptoms except occasional mild pruritus. The patient was in good health and was on no medications. Physical examination revealed multiple violaceous to brown, indurated, 5-50-mm, round to oval plaques on the face, arms, shoulders, and back (Fig. 1), as well as a solitary lesion on the right thigh. Surface telangiectases were noted, especially on the shoulder lesions. There was no scaling or ulceration. Routine laboratory tests were unremarkable. In April 1999, another medical center performed a biopsy of what they thought was sarcoidosis. The results were reported as "possible angiolymphoid hyperplasia with eosinophilia." With the possibility of granuoma faciale (GF) in mind, another skin biopsy was obtained from a facial lesion. This revealed a diffuse, relatively dense infiltrate of neutrophils, eosinophils and mononuclear inflammatory cells in dermis with an obvious Grenz zone (Fig. 2). Pilar units were intact, and endothelial cell swelling was present (Fig. 3). Retrospective evaluation of the initial biopsy, taken from the back, revealed the same changes, and helped confirm the diagnosis of GF. The patient was treated with liquid nitrogen for 20 s followed immediately by intralesional triamcinolone acetonide (5 mg/ml). This treatment was repeated every 4 weeks for three courses, resulting in partial resolution of the lesions.     

Generalized linear porokeratosis

A 23-year-old woman was seen for widespread skin lesions present since the age of 2.5 years. Twenty years ago, she developed a brown macular lesion on her right buttock. The lesion became hyperkeratotic and subsequently spread through the posterior aspect of her right leg. It later spread to the right side of the trunk and to the right arm. When she was 9 years old, she developed similar lesions on her left arm and leg. After she was 13 years old, no new skin lesions appeared. There was no family history of similar lesions. On examination, there were numerous linear and whorled, reddish-brown, hyperkeratotic plaques, with central atrophy and raised borders, following Blaschko's lines on all of the extremities. These lesions on the extremities extended to the dorsum of the hands and feet (Fig. 1). She had hyperkeratotic lesions on the pressure points of both of the soles, but no palm involvement. The number of lesions on the right side was greater than that on the left. Reddish-brown annular plaques with central atrophy and raised borders, appearing in zosteriform configuration, and numerous individual 2-3-mm erythematous lichenoid papules were observed on the right side of the thorax and the right inguinal region (Fig. 2). No face, scalp, or mucous membrane involvement was seen. The nails of the second and fifth fingers of the right hand and the nail of the third finger of the left hand showed nail dystrophy with longitudinal ridges and pterygium. All the nails of the right foot and the nails of the first and fifth toes of the left foot showed dystrophic changes with subungual keratosis. The patient was otherwise in good health. Two biopsy specimens taken from a hyperkeratotic plaque and a lichenoid papule showed an epidermal invagination with angulated parakeratotic tier, denoting cornoid lamella. The epidermis just underneath the cornoid lamella displayed vacuolization and the granular layer was absent. The adjacent epidermis was atrophic, and hydropic degeneration within the basal cell layer was seen. In the dermis, a nonspecific, mild, chronic, inflammatory cell infiltrate, telangiectatic vessels, and pigment-laden macrophages were present. These findings were consistent with linear porokeratosis (Fig. 3). Microscopic examinations and mycologic cultures of the nails were negative. We decided to treat our case systemically with retinoids, but the patient refused this therapy. So, topical tretinoin 0.05% was started once a day. A marked improvement was observed in hyperkeratosis through the first 4 weeks of treatment and plateaued at 8 weeks. After 10 weeks, the lesions had almost disappeared. We planned to continue the applications every other day. One year later, she remains stable with application of topical tretinoin 0.05% twice a week and is satisfied with the final appearance. She is under regular follow-up.     

Granular parakeratosis

A healthy 62-year-old woman was referred to our dermatology department with a 1-month history of a pruritic axillary eruption. On examination, multiple erythematous and brownish hyperkeratotic papules were seen in both axillae. Some of these lesions coalesced into plaques, with small areas of sparing, and a background erythematous color was also found in the axillary vaults (Fig. 1). There was no involvement of other intertriginous sites and there were no associated systemic symptoms. The patient was not obese. The patient had removed the hair from her axillae with wax 3 weeks before the development of the eruption. Moreover, she had changed her antiperspirant 1 week before the onset of the lesions. A cutaneous biopsy for histologic analysis was performed. Histologically, the stratum corneum was thickened, with persistent nuclei together with countless small basophilic granules. The granular layer was preserved and, in some areas, hypergranulosis was found (Fig. 2). These findings were characteristic of granular parakeratosis. The cutaneous lesions resolved completely after 1 week of treatment with topical betamethasone dipropionate and gentamicin sulfate (twice daily). The patient was urged to discontinue her use of deodorants.     

Squamous cell carcinoma in a patient with Netherton's syndrome

A 29-year-old white woman with a history of Netherton's syndrome presented with two squamous cell carcinomas on the right dorsal hand and the left upper arm. She reported a 2-year history of these lesions, which were originally treated as warts. She denied excessive sun exposure, immunosuppressive therapy, or a previous history of skin cancer. Her past medical history included acute renal failure, multiple urinary tract infections, meningitis, and recurrent otitis media as a child. In addition, she had an ovarian abscess at 4 years of age with resulting salpingo-oophorectomy. She also reported a history of severe myopia, glaucoma, and multiple ocular infections with a resulting corneal scar. In addition to atopic dermatitis, she had a 10-year history of psoriasis. Her medications included topical steroids and emollients for atopic dermatitis and psoriasis, in addition to Timolol ophthalmic drops for glaucoma. Her family history was significant for a 22-year-old sister with Netherton's syndrome (Fig. 1). She denied any history of skin cancer in her sister or other members of her family. On physical examination, she had an exfoliative erythroderma, madarosis, and diffuse patchy alopecia. In the bilateral axilla, she had well-defined pink scaly plaques which were confirmed as psoriasis by biopsy. On the right dorsal hand, she had a 1.5 x 1.0 cm pink verrucous plaque (Fig. 2). On the left upper arm, she had a 1.5 x 0.8 cm pink scaly plaque. Biopsies of both sites confirmed squamous cell carcinomas. Both lesions were completely excised with 4 mm margins.     

Conjunctival involvement in familial chronic benign pemphigus (Hailey-Hailey disease)

A 25-year-old woman with a 1-year history of malodorous, red, and eroded lesions in frictional sites was seen in the dermatology department. She did not give a family history of a similar condition. She had an 18-month-old baby and her initial complaint was of genital pruritus with profuse vaginal discharge which appeared within 2 months of delivery. She was treated for candidal vaginitis but pruritus persisted and skin lesions occurred on both sides of the groin. The lesions subsided as long as the local treatment for candidal vaginitis was continued, but recurred more than three times in a year, each time with additional and more severe lesions involving the axillae, retroauricular sites, and scalp. She received no specific treatment during this time and her complaint about the eyes was the reason she was referred to a dermatologist. On dermatologic examination, impetigo-like, serpiginous plaques with severe erythema, erosions, and an active border were observed behind the ears, in the axillae, and in the pubis; these tended to expand peripherally and were partly covered with greasy yellowish scales particularly on the scalp. Satellite pustules and vesicles were observed at the adjacent sites (Fig. 1). Conjunctivae were bilaterally hyperemic and fine yellowish scales were also apparent on the palpebral rim (Figs 2 and 3). Ophthalmologic examination revealed severe conjunctival oedema and ulcerating blepharitis. Cornea appeared normal and vision was unimpaired. Results of Schirmer's test (15 and 17 mm) were within the normal range of tear secretion without an anaesthetic. Swab cultures from the peripheral pustules and erosions showed S. aureus infection while Sabourraud culture remained negative. Skin and conjunctiva biopsies for direct microscopy and immunofluorescence were performed. Histopathologic features were identical in either specimen disclosing suprabasal blister formation, acantholysis, and villus-like protrusions lined by a single layer of basal cells (Fig. 4). IgG, IgA, IgM, and complement C₃ immunofluorescence was not detected at the epidermis of the skin adjacent to serpiginous lesions. A direct immunofluorescent test of conjunctival biopsy also remained negative. The patient was given clarithromycine 500 mg/day for 10 days and topical treatment was also applied. A rapid response was obtained within the first week of treatment.     

Keratosis lichenoides chronica in an Indian child following erythroderma

A 4-year-old boy presented with mildly itchy, linear, skin lesions over the trunk, arms, and face of 3 months' duration. He had previously been admitted to a private hospital for generalized exfoliation of the skin following drug intake for fever and throat pain. The nature of the drugs was not known. The exfoliative dermatitis was treated with oral prednisolone, 10 mg daily, tapered over 3 weeks. No further topical or oral medication was given. The present skin lesions started 1 month after the cessation of the steroids. There was no family history of skin lesions, voice changes, or systemic complaints. Cutaneous examination showed multiple violaceous, linear, reticulate ridges with adherent scaling over the chest, back, and neck. There were scaly, flat-topped papules over the extensor aspects of both upper arms and the buttocks, and scaly plaques over the cheeks (Figs 1a-d and 2a,b). The scalp showed diffuse greasy scaling. There were no oral, genital, axillary, or eye lesions. The nails were normal. Systemic examination did not reveal any abnormal finding. Routine hematologic investigations, liver and kidney function tests, tests for hepatitis B and C, and enzyme-linked immunosorbent assay (ELISA) for HIV were normal. Histopathology from skin lesions on the back revealed hyperkeratosis, patchy parakeratosis, follicular plugging, alternating irregular acanthosis and epidermal thinning, basal cell degeneration, and a band-like inflammatory infiltrate of lymphocytes, histiocytes, and a few plasma cells (Fig. 3). Based on the classical clinical features and histopathology, keratosis lichenoides chronica was diagnosed, and topical 1% hydrocortisone acetate cream, twice daily, was prescribed. There was slight relief of pruritus at a follow-up visit after 3 weeks; however, the patient was subsequently lost to follow-up.     

A case of recurrent Sweet's syndrome in an 80-year-old man: a clue to an underlying malignancy

An 80-year-old man presented with multiple erythematous papules on the trunk and extremities of a few weeks' duration. He had no past medical or family history of skin diseases or any other medical diseases. A biopsy showed a perivascular lymphohistiocytic infiltrate and sparse neutrophils with several atypical lymphocytes in the deeper dermis. With an initial diagnosis of T-cell pseudolymphoma or unspecified neutrophilic dermatosis, he showed a brisk response to an intramuscular injection of triamcinolone acetonide (40 mg/mL). After 1 month, his skin lesion recurred. Steroid was given with a good clinical response. One month later, however, his skin lesion relapsed. At this time, he presented with disseminated pustulopapular lesions on the trunk and extremities. Examination revealed multiple, variable-sized, erythematous plaques with central pustules on the extremities (Fig. 1). The mucous membranes were not involved. He had no pain or tenderness. He had no systemic symptoms. Laboratory tests showed a hemoglobin level of 10.3 g/dL, a leukocyte level of 6,900/mm(3), with an increased proportion of segmented nuclear neutrophils (83%), and an elevated C-reactive protein. A skin biopsy revealed a dense perivascular and interstitial infiltrate composed of neutrophils with marked dermal edema (Fig. 2). Sweet's syndrome was the final diagnosis and he was treated with oral prednisolone (30-40 mg/day) and dapsone (50 mg/day) for 2 months. As this 80-year-old patient had a recurrent history of similar skin lesions and anemia, an underlying hematologic malignancy was suspected. A bone marrow biopsy showed typical myelodysplastic syndrome (MDS). The hemoglobin level was decreased to 5.3 g/dL during a follow-up period of 5 months. The skin lesions recurred despite oral steroids and dapsone. The patient received only symptomatic treatment, such as a transfusion, for the underlying malignancy MDS.     

Basement membrane changes in psoriatic patients on long-term topical corticosteroid therapy

It has been observed that the beneficial anti-inflammatory effects of topical steroids in psoriasis are counteracted by increasing instability of the disease, with rebound phenomena associated with the cessation of these drugs. We report the occurrence of multi-layered fragmentation and disorganization of the basal laminae in active, untreated psoriatic lesions, resolving and uninvolved, inadvertently steroid-treated psoriatic skin, as well as in a variety of non-psoriatic dermatoses treated with fluorinated topical steroids for prolonged periods. These changes, which were associated with a moderate to severe loss of dermal collagen, were not found in untreated and treated psoriatic controls, with or without concomitant alpha 1-antitrypsin deficiency, who had not received steroids, suggesting that they were probably the consequence of prolonged fluorinated steroid use. This conclusion is supported by the observation that the largest number of layers (10-15) of fragmented basal laminae was noted in the patients who had received fluorinated steroids for 6 years or more, while those on 4 years or less of fluorinated steroid therapy exhibited only three to seven layers of fragmented basal laminae. In psoriatic lesions, the fragmentation of the basal lamina was associated with the presence of basal keratinocyte herniations (BKH), the frequency of which has been shown to parallel clinical psoriatic activity. The persistence of these electron-microscopic markers of psoriatic activity (i.e. BKH) in psoriatic plaques treated with prolonged fluorinated steroids suggests that loss of integrity of the basement membrane, as indicated by the presence of multi-layered fragmentation of the basal lamina, may account for the instability of the psoriatic lesions treated with prolonged topical fluorinated steroids.     

Primary malignant melanoma of the oral cavity: case report

An 80-year-old-female patient had a pigmented lesion on: the hard palate, the soft palate, the alveolar mucosa and the vestibular mucosa of the maxillary gingiva. Pigmented macules and patches had been persistent and asymptomatic for many years (Fig. 1). The lesion exhibited irregularities of pigmentation, border and surface contour. About 1 year later the patient had noticed an extension of the pigmented macules and plaques; there was also the appearance of nodules of the maxillary gingiva accompanied by swelling. Loosening of teeth as a result of extensive destruction of bone was further noted (Fig. 2). The histological examination showed a downward streaming in the dermis of the tumor cells and a disintegration and ulceration of the epidermis (Fig. 3). An increased number of large round or polygonal cells resembling atypical epithelioid cells were found on the submucosa. The atypical cells had enlarged, pleomorphic nuclei with prominent and sometimes multiple nucleoli. Mitoses were observed at various tissue levels (Fig. 4). Abundant pigmented melanin was present in the tumor cells (Fig. 5). Many cells had fine, dusty melanin particles. The tumor cells showed great variations in size. Immunohistochemical staining, with S100 protein and HMB45 antibodies, stained many of the spindleshaped cells, indicating that they were melanocytic cells. An inflammatory infiltrate of lymphocytes was seen in a band beneath the invading tumor cells.     

Lichen planopilaris.

P, a 20-year-old laborer displayed initial symptoms of the disease in question when he was 10 years old. Initially he had an asymptomatic progressive loss of hair on the scalp. A couple of years later he had mild to moderate pruritis, and the appearance of slate-blue eruptions on the scalp and elsewhere on the body. This resulted in a complete loss of hair on the vault of the scalp, which led him to seek specialist opinion. Skin surface examination revealed the presence of grayish-blue acuminate follicular papules, disposed singly and in groups (plaques). The pilo-sebaceous orifices were conspicuously obliterated and filled by keratin plugs. Perifollicular erythema was a predominant feature on the scalp. The lesions were present over the scalp, around the neck, chest, back, axillae, groin and legs. Shiny atrophied scalp skin depicting scarring alopecia mimicking male-type baldness was a salient feature. In addition, it was studded with conspicuous acuminate papules in its center (Fig. 1a). The known nonhairy (glabrous) skin had classic lichen planus lesions (Fig. 1b). Hemotoxylin-eosin stained microsections prepared from typical lichen planus (LP) lesions over the abdomen and those of lichen planopilaris (LPP) of the scalp were simultaneously studied. The former revealed changes in the epidermis comprising of hyperkeratosis, increase in thickness of stratum granulosum, hydropic degeneration of the basal cell layer and band-like lympho-histiocytic infiltrate pressing against and invading the epidermis, while the latter revealed uniform atrophy of the epidermis and vacuolization of basal cells. The hair follicles were dilated and were filled with keratin plugs. In addition to fibrosis of the dermis, pigment laden microphages and lympho-histiocytic infiltrate was prominent. The follicles and the sebaceous glands were absent. However, arrectores pilorum and sweat glands were preserved (Fig. 2a,b).     

A case of idiopathic granulomatous cheilitis and vulvitis

A 36-year-old woman presented with chronic recurring dermatitis of the vulva, perineum, and lips. The genital lesions had a 3-year history and were associated with slight pruritus and occasional pain. The lesions of the upper lip had a history of 6 months. The patient had been treated with cycles of antimycotics and topical steroids which only partially controlled the symptoms during treatment. Dermatologic examination showed erythematous, infiltrative dermatitis with edema of the labia majora and persistent edema of the upper lip (Fig. 1a,b). Routine blood chemistry, urine analysis, and chest X-ray were normal. Microscopic examination and cultures of vaginal swabs did not reveal any pathogenic bacteria or fungi. Histologic examination of a biopsy of vulval lesional skin showed lichenoid lymphocytic infiltration of the papillary dermis and small, nonnecrotic epithelioid granulomas in the deep dermis (Fig. 1c,d). No microorganisms, including acid-fast bacilli or fungi, were identified. Culture was negative for fungi. Polymerase chain reaction was negative for the mycobacterial genome. Histologic examination of a biopsy from the upper lip showed similar results. The pathology reports of both regions were compatible with a diagnosis of granulomatous cheilitis and vulvitis. To investigate concomitant asymptomatic inflammatory bowel disease, the patient underwent colonoscopy with retrograde ileoscopy and gastroscopy, which were both negative. The patient refused radiographic examination of the small intestine with a barium meal. The patient was treated with systemic metronidazole (500 mg/day). After 6 months of therapy, the upper lip showed significant improvement and erythema and desquamation in the genital area showed slight improvement, but genital edema was unaffected.     

LEPROMATOUS LEPROSY WITH EXTENSIVE TRUNCAL SEBORRHEIC KERATOSES AND ACRAL VERRUCA VULGARIS

A 37-year-old man came to us with asymptomatic dark colored warty iesions of 3V2 years' duration. The lesions started on the dorsa of the feet and hands and progressively increased in size and number to cover more than 60% of these areas. Later, they spread to other parts of the body. There was no history of sudden loss of weight or appetite, change in bowel habits, or of major illness in the past. The patient had not taken any medication. A general and systemic examination of the patient revealed no abnormality. On dermatological examination, hyperpigmented well-defined, verrucous papules and plaques of varying sizes were seen on the extremities, almost covering the dorsa of the hands and feet (Figs. 1–3). Small flattopped hyperpigmented papules with a smooth surface and “stuck-on” appearance were present on the trunk (Fig. 4). The rest of the body had scattered hyperpigmented papules and plaques, up to 2 × 2 cm in size with a minimally verrucous surface. The patient also had a shiny appearance of the face, infiltration of the ear lobes, with mild ichthyosis of the limbs. Examination of the peripheral nerves revealed bilateral thickening without tenderness or sensory loss. Slit smears for acid-fast bacilli from the earlobe and normal skin showed a bacteriological index of 5+ and 2+, respectively. A diagnosis of lepromatous leprosy with extensive verruca vulgaris and seborrheic keratoses was made. Biopsies of the normal-looking skin, the flat truncal lesions, and the verrucous lesions on the extremities were taken. Histopathologic examination of the trunk lesions showed hyperkeratosis and acanthosis with interwoven tracts of basaloid cells and horn cysts, indicative of the acanthotic type of seborrheic keratosis (Fig. 5). Below the epidermis there was a clear zone of dermis. A foamy macrophage granuloma was seen in the deeper dermis; M. leprae with Fite-Faraco staining was demonstrated. On histopathologic examination the acral lesions showed hyperkeratosis, acanthosis, and papillomatosis. The rete ridges were elongated and bent inwards and typical vacuolated cells were seen in some areas, indicative of verruca vulgaris (Fig. 6). The biopsy from a lesion on the arm revealed seborrheic keratosis and verruca vulgaris in continuity along the epidermis with a foamy macrophage granuloma in the lower dermis (Fig. 7). Mycobacterium leprae were demonstrable in all the sections. Thus, the histopathology of the lesions confirmed a diagnosis of lepromatous leprosy with acral verruca vulgaris and truncal seborrheic keratosis. The patient was further investigated and baseline parameters including urine and stool examination, hemogram, blood sugar, and renal and hepatic function tests revealed no abnormality. Keeping in mind the sign of Leser-Trelat, the patient was also investigated for any focus of malignancy. Gastroscopy, x-ray of the chest, and other tests were normal. The Mantoux and blood serology for H(v were negative. The patient was started on antileprosy treatment according to the WHO Multidrug Regimen and was also given a course of levamisole. Minimal response was noted. Eighteen months later the patient returned to the hospital and a 50% regression of the acral lesions was seen. A few lesions were also cauterized by electrocautery and the response was good.