Crusted Norwegian scabies, an opportunistic infection, with tocilizumab in rheumatoid arthritis

Background

Crusted Norwegian scabies is an extremely rare hyperkeratotic variant of scabies infestation. We report herein a case of crusted scabies in a woman with severe rheumatoid arthritis (RA) treated by tocilizumab (TCZ), a monoclonal antibody blocking the interleukin-6 receptor.

Observation

An 80-year-old female with severe RA developed erythroderma followed by hyperkeratosis, widespread scaling over the trunk, arms, hands and limbs, and crusted lesions on her scalp. She was on TCZ (8 mg/kg per month) with prednisone (20 mg/d). Several hypotheses were evoked (i.e., vasculitis, psoriasis or paraneoplasic syndrome) but a microscopic examination of the skin surprisingly demonstrated numerous scabies mites and eggs leading to the diagnosis of crusted (Norwegian) scabies. After repeated use of ivermectin and application of topical piperonyl butoxide cream, scabies disappeared. However, there had been outbreak of scabies among two individuals in the staff and two patients. TCZ was stopped after four infusions because of a severe infectious pneumonia and failure.

Conclusion

Crusted scabies should be evoked in case of pruritus or erythroderma occurring under TCZ therapy and it may be explained by the interleukin-6 blockade.     

Psoriatic erythroderma associated with enalapril

A 59-year-old man with a 35-year personal and positive family history of psoriasis was admitted to our department for treatment of psoriatic erythroderma. The patient had commenced therapy with enalapril 10 mg b.i.d. for the treatment of hypertension approximately 6 weeks before hospitalization. Five weeks after the initiation of enalapril, his psoriasis began to flare, and for a period of about 1 week it reached the extent of erythroderma. The patient did not associate the psoriatic flare with other factors such as infections, trauma, or stress. The patient presented with diffuse erythema and pronounced desquamation covering his entire trunk, scalp, and extremities (Figure). Nearly 100% of the body surface area was involved. The palms and soles were also affected, displaying erythema, hyperkeratosis, and painful fissures. The nails showed pits, oil spots, and subungual hyperkeratosis. The patient also had psoriatic arthritis affecting the interphalangeal joints of his fingers. Laboratory tests revealed an elevated erythrocyte sedimentation rate, an elevated creatinine level of 180 mmol/L, a blood urea nitrogen level of 10.8 mmol/L, and a uric acid level of 716 mmol/L. Urinalysis showed proteinuria of 1.5 g/24 h. The patient's renal condition was diagnosed as chronic tubulointerstitial nephritis, most probably related to his dermatologic disease. Allopurinol and dietary measures were recommended. Following treatment with methotrexate and replacement of enalapril therapy, the erythema and scaling gradually subsided and became confined to his pre-eruptive chronic plaques (approximately 5% of body surface area). Rechallenge with enalapril was not performed.     

Bullous-hemorrhagic Darier disease

A 48-year-old man presented with a 4-month history of papular hyperkeratotic diffuse lesions on his trunk, arms, and neck that were highly pruritic (Figure 1). He also had "V"-shaped nicks in the nails, mucous white papules on his palate, and diffuse desquamation on the scalp. Abnormal laboratory values included elevated levels of uric acid and triglycerides. Serum electrolytes, blood sugar, and renal and liver function test results were within normal range. X-ray film and abdominal ultrasonography findings were also normal. Histopathologic study of the biopsy from the thorax revealed acantholysis with suprabasal clefting, intraepidermal lacunae, and dyskeratosis with corps ronds. The clinical features and results of the histopathologic studies suggested a diagnosis of Darier disease (Figure 2), but the course was not typical of this entity because the patient had no family or personal history of previous cutaneous lesions and the age of onset was older than usual. In the course of the disease, he developed blisters and small black hemorrhagic macules with jagged borders on the back of his hands (Figure 3). Nikolsky's sign was negative. A biopsy of a blister was performed, which confirmed Darier disease, studied by means of immunofluorescence. Measurement of porphyrins in the urine was also ordered. Direct immunofluorescence did not show deposition of immunoglobulins or complement, and the study of porphyrins was normal. The patient was treated with an oral retinoid (acitretin 10 mg daily), but treatment was stopped because he developed an increase in triglycerides; therefore, control of the disease with oral antihistamines, 5-fluorouracil 1% cream, and topical tazarotene was used, with mild improvement.     

Segmental lesions in pityriasis rosea: a rare presentation

A 34-year-old nonpregnant woman noted a mildly pruritic skin lesion on her right breast for 1 week following an episode of coryza, malaise, and low-grade fever of 3 days' duration. The latter symptoms subsided without any treatment. Ketoconazole cream prescribed by her family physician did not resolve the breast eruption after 1 week, so was stopped. A week later, multiple skin lesions erupted suddenly on her right chest wall starting near the herald patch in midaxillary line and spread distally until the midback. She then consulted the authors. She denied a history of abrasion or trauma to the affected areas. Travel, contact, sexual, and drug histories were unremarkable. She categorically denied past or family history of eczema, psoriasis, contact dermatitis, and drug eruptions. On examination, the initial lesion was an annular and well-demarcated erythematous plaque on her right breast in the upper outer quadrant. Multiple small oval scaly plaques were noted, extending distally along the ribs to the midline on her back, not crossing the midline, predominantly over the distribution of right T4 dermatome (Figure 1). Peripheral collarette scaling was noted (Figure 2). A few scattered small lesions were also noted in the vicinity of this dermatome. Three isolated small plaques were also present on the trunk, one in the supramammary area and the other two on the abdomen and back, respectively. Palmoplantar and mucosal surfaces were uninvolved. The rest of the skin and systemic examination revealed no abnormalities. Complete blood cell counts, fasting glucose, and urinalysis were normal. Venereal Disease Research Laboratory (VDRL) results were nonreactive and human immunodeficiency virus (HIV) antibodies were negative. Repeat testing ofVDRL in serial dilutions and HIV antibodies after 3 months were also nonreactive and negative, respectively. Scrapings from the initial large lesion and subsequent smaller eruptions did not show any evidence of fungal infection on potassium hydroxide smear examination. The patient declined skin biopsy; however, we thought that the most diagnostic label for this condition was pityriasis rosea. Hence, we treated her with triamcinolone acetonide ointment 0.025% to be applied twice daily and desloratadine tablet 5 mg daily for 10 days. The patient demonstrated complete resolution, leaving postinflammatory hypopigmentation. There was no recurrence until 1 year after complete remission.     

Norwegian Crusted Scabies: An Unusual Case Presentation

Scabies is a contagious condition that is transmitted through direct contact with an infected person and has been frequently associated with institutional and healthcare-facility outbreaks. The subtype Norwegian crusted scabies can masquerade as other dermatologic diseases owing to the heavy plaque formation. Successful treatment has been documented in published reports, including oral ivermectin and topical permethrin. Few case studies documenting the treatment of Norwegian crusted scabies have reported the use of surgical debridement as an aid to topical and/or oral treatment when severe plaque formation has been noted. A nursing home patient was admitted to the hospital for severe plaque formation of both feet. A superficial biopsy was negative for both fungus and scabies because of the severity of the plaque formation on both feet. The patient underwent a surgical, diagnostic biopsy of both feet, leading to the diagnosis of Norwegian crusted scabies. A second surgical debridement was then performed to remove the extensive plaque formation and aid the oral ivermectin and topical permethrin treatment. The patient subsequently made a full recovery and was discharged back to the nursing home. At 2 and 6 months after treatment, the patient remained free of scabies infestation, and the surgical wound had healed uneventfully. The present case presentation has demonstrated that surgical debridement can be complementary to the standard topical and oral medications in the treatment of those with Norwegian crusted scabies infestation.     

Hyperkeratosis of the heels: treatment with salicylic acid in a novel delivery system

A 43-year-old woman presented with dryness and scaling of the lateral and posterior aspects of both heels, which was diagnosed as hyperkeratotic xerosis (Figure 1). Pertinent medical history included dry skin with winter exacerbation and painful hyperkeratosis of the heels present for many years. The patient applied a topical multivesicular cream formulation of 6% salicylic add (Salex, Healthpoint Ltd., Fort Worth, TX) to one foot b.i.d. The physician was blinded as to which foot was treated. After 2 weeks of treatment, it was apparent that the patient was applying the cream to the right foot, as evidenced by reduced dryness, scaling, and hyperkeratosis (Figure2). The patient continued treatment of the same foot for an additional 2 weeks, revealing a dramatic improvement of the right heel,which appeared smooth and soft and devoid of pain. No irritation was associated with treatment; the patient commented that this was the best her heel had been "in years." Subsequently, the patient treated both heels with salicylic acid 60%, multivesicular cream. A second patient, a 25-year-old woman, was treated for ichthyosis vulgaris and hyperkeratosis of both heels. She presented w ith multiple painful fissures and hyperkeratosis of the posterior heels bilaterally (Figure 3). After I week of topical treatment with salicylic add 6%, multivesicular cream applied b.i.d. to the left heel only, there was rapid resolution of both hyperkeratosis and pain (Figure 4).     

An unusual cutaneous manifestation of Crohn's disease

A 61-year-old man with a 12-year history of quiescent Crohn's disease on mesalamine presented to his gastroenterologist in April 2009, complaining of abdominal cramping, diarrhea, and a 25-lb weight loss over 6 weeks. He did not respond to prednisone 50 mg and 6-mercaptopurine 100 mg daily. Abdominal computed tomography findings revealed diffuse submucosal edema consistent with extensive colitis. Colonoscopy demonstrated diffuse inflammation with erythema, friability, and shallow ulcerations in the rectum and colon. Biopsies were consistent with Crohn's colitis. He was admitted for infliximab infusion for his unremitting diarrhea. Five days before admission, the patient noted mild swelling and redness of the left lower eyelid, which progressed to involve the right lower eyelid with frank pus draining from both eyes. He had no visual impairment or eye pain. Two days before admission, an ophthalmologist prescribed a steroid eyedrop with no relief. He also complained of seropurulent painful skin lesions on his face and scalp, which spread to involve his upper trunk and proximal arms. On admission to the hospital, dermatology, ophthalmology, and infectious disease consultations were obtained to rule out disseminated infection before initiation of infliximab therapy. The patient was afebrile and hemodynamically stable. His oral mucosa was normal. He had prominent bilateral lower eyelid edema, erythema, and superficial erosions with hemorrhagic crusting and frank green purulent drainage from both eyes, with crusting along the lower lash line and bilateral sclera injection (Figure 1). On his scalp, face, trunk, and proximal extremities, he had 25 to 30 erythematous, 4- to 8-mm papulopustules with narrow red halos, some with central necrosis and crusting (Figure 2). Cultures from the purulent ocular drainage and pustules on the trunk and arms were all negative for bacteria, virus, and fungi. Gram stain from the eye drainage showed polymorphonuclear leukocytes without organisms. Tissue cultures were negative for bacterial, fungal, and mycobacterial infection. Skin biopsy taken from the central upper back demonstrated subcorneal pustules with areas of eroded epidermis and collections of neutrophils in the superficial dermis (Figure 3). Special stains were negative for organisms. He received infliximab infusion 5 mg/kg for a total dose of 420 mg over 2 hours. Within 48 hours of infusion, there was notable decrease in size of lesions, in addition to reduction of purulent drainage from both eyes. The patient was discharged home following infliximab infusion. His skin lesions resolved during a period of 2 weeks, leaving small pink atrophic scars. He received his second infusion of infliximab 2 weeks after discharge with continued improvement in his gastrointestinal symptoms.     

Occlusive irritant dermatitis: when is "allergic" contact dermatitis not allergic?

CASE 1: A 38-year-old teacher presented with a 3- to 4-week history of a linear, erythematous, vesicular, and pruritic eruption of her left wrist. She had been wearing a new elastic bracelet for 4 weeks before the onset of her eruption. Although there was no history of allergy to rubber products or jewelry, an allergic contact dermatitis to rubber was suspected. Patch testing to rubber chemicals and the elastic bracelet revealed no reactions at 48, 72, and 96 hours. She stopped wearing the bracelet and used a corticosteroid cream with rapid resolution of the problem. The patient resumed wearing the bracelet, and there has been no recurrence in the past 2 months. CASE 2: A 12-year-old boy presented with a 1-month history of an itchy, scaly, erythematous 1-cm patch over the midline of his lower lip. The patient complained of tiny blisters initially with persistent erythema, mild scaling, and associated pruritus. The patient plays the saxophone and he had been practicing more intensely (3 to 4 h/d) for a musical competition. Allergic reaction to his wood reed was suspected, but patch testing with a moistened portion of his reed and reed shavings in a drop of water revealed no reaction at 48 and 72 hours. Treatment with hydrocortisone 1% cream bid for 3 days led to complete resolution of the dermitis and pruritus. Playing the saxophone 1 h/d has not led to any recurrence. CASE 3: A 33-year-old woman presented with erythema, scaling, and pruritus of 1 month's duration beneath her engagement and wedding rings, which were worn together on her left fourth finger (Figure 3). Although she had no history of previous sensitivity to earrings, watch clasp, blue jean rivets, or other jewelry, allergic contact dermatitis to nickel was suspected. Patch testing was performed to the common metal allergens nickel, cobalt, chromium, and gold. Readings at 48 hours and 1 week revealed no positive reactions. The patient wore her rings on the right hand for 1 week and used fluocinonide 0.5% cream twice daily for 1 week with resolution of the dermatitis. She has subsequently begun wearing the rings again on her left hand with care to dry her hands and rings after washing, and there has been no recurrence of her dermatitis.     

Granuloma faciale with extrafacial lesions

A 35-year-old man presented with a 7-year history of gradually enlarging plaques on his face and trunk. The first lesions had developed on both sides of the forehead and the left cheekbone (Figure 1). Four years later similar lesions appeared on his neck and back. He presented a histologic report of a biopsy specimen from a facial plaque performed 5 years earlier that was diagnostic for granuloma faciale. He had different treatments such as topical steroids and cryotherapy without improvement. The appearance of new lesions on his trunk and the gradual enlarging of the old lesions convinced the patient to seek further treatment. Physical examination revealed dusky, violaceous plaques and papules, 0.5 to 2 cm, well-circumscribed, slightly elevated, and located on the face and trunk, with mild pruritus (Figure 1 and Figure 2). Laboratory investigations, including complete blood cell count, VDRL test, antinuclear antibody test, biochemical parameters, and chest x-ray, did not reveal any abnormalities. A skin biopsy taken from the upper part of the back showed similar features to the facial lesion, detected 5 years before, revealing a dense, polymorphous infiltrate involving mid and deep dermis and displaying a diffuse and perivascular pattern (Figure 3A). A narrow grenz zone of normal collagen was consistently observed between dermal infiltrate and epidermis as well as around the pilosebaceous follicles (Figure 3A). The infiltrate mainly consisted of eosinophils and lymphocytes, but neutrophils (often displaying leukocytoclasis), macrophages, and plasma cells were also present (Figures 3B, 3C). Some mast cells were also identified by staining with toluidine blue (Figure 3D). Perivascular infiltrates were often seen, sometimes penetrating vessel walls and in association with leukocytoclasis. Hyalinization of vessel walls, extravasation of red blood cells around capillaries, and nuclear dust were also noted. The epidermis did not show any remarkable change except for slight acanthosis. A diagnosis of granuloma faciale with extrafacial lesions was made, and a systemic therapy with hydroxychloroquine (200 mg twice daily for 6 weeks) was recommended.(1,2).     

Vesicular palmoplantar pityriasis rosea

A 16-year-old young man presented with intensely itchy erythematous dermatitis on the body for 1 week and vesicular lesions on the palms and soles for 4 to 5 days. Lesions on the palms and soles were accompanied by severe burning and itching. The patient gave a history of sore throat and fever, 1 week prior to the onset of lesions. A general physical examination was normal, and cutaneous examination revealed multiple, well-defined erythematous scaly plaques with collaret scaling on the trunk and extremities (Figure 1). Vesicular lesions were seen on the palms and soles (Figure 2). The differential diagnoses we considered were pityriasis rosea and secondary syphilis. The possibility of dermatophytid, vesicular pityriasis rosea, and pompholyx was limited to the palms and sole lesions. Complete blood cell count was within normal limits. Results from antistreptolysin O titer, potassium hydroxide mount, and venereal disease research laboratory were negative. Skin biopsies were taken from the back and left palm. The biopsy specimen from the back revealed focal spongiosis, lymphocyte exocytosis, vacuolar changes in the basal layer, and perivascular lymphocytic infiltrate in the dermis (Figure 3). The biopsy obtained from the vesicular lesion on the left palm revealed an intraepidermal vesicle with no evidence of acantolytic process (Figure 4). A diagnosis of pityriasis rosea was made and the patient was started on clarithromycin 500 mg once a day for 7 days, along with antihistamines and emollients. The lesions faded dramatically in a very short period, and there was significant involution of almost all of the lesions after 7 days of clarithromycin. During the 6 months of follow-up, no recurrence was observed.     

Food-induced acute generalized exanthematous pustulosis in a pregnant woman

A 21-year-old woman of Romany origin, in the third trimester of her fourth pregnancy, was admitted to the hospital because of a generalized erythematous and pustular eruption and desquamation involving her face, neck, trunk, and extremities. The skin changes were accompanied by fever (100.4 degrees F [38 degrees C]) and malaise. The patient was convinced that the dermatitis was induced by the consumption of "spoilt" pork sausage (bad smell, changed taste) approximately 24 hours earlier. Clinical examination revealed a woman with phototype III skin, black eyes, and black hair, in good general health. Widespread, symmetrical, moderately intense erythema and isolated or coalescing targetoid lesions studded with discrete, pinhead-sized, nonfollicular pustules in the center or at the periphery were distributed over her face, trunk, groins, and upper and lower extremities (Figures 1). On the neck and abdomen, lamellar desquamation was observed (Figure 2). Palms, soles, scalp, mucous membranes, hair, and nails were not affected. Nikolsky's sign was negative. The patient complained of very slight skin burning and itching. The pregnancy was proceeding without any complications and her obstetric status was normal. The woman had neither any accompanying diseases, nor previous personal or family history of psoriasis, nor any known allergies. She had taken no systemic medication (not even vitamins). She had three pregnancies; two ended with the delivery of healthy babies and one of them was aborted at her will. Laboratory studies revealed leukocytosis (13.2 x 109/L), neutrophilia (8 x 109/L), anemia (hemoglobin, 108 g/L), and an elevated erythrocyte sedimentation rate (68-110 mm/h). The results from the following investigations were normal: urinalysis, renal and hepatic function, serum albumin, Ca, Na, K, aspartate aminotransferase titer, cryoprotein, hepatitis B surface antigen, and serum markers for syphilis. Bacterial and fungal cultures of pustular content were sterile. A skin biopsy specimen of lesional skin revealed subcorneal pustules containing leukocytes and necrotic keratinocytes and a mixed perivascular inflammatory infiltrate with isolated eosinophils in the dermis (Figure 3). The patient was treated with systemic methylprednisolone in gradually reduced doses, fluocinonide cream 0.05%, and emollients. As a result, her fever disappeared and her erythema faded. Frequent obstetric examination and cardiotocography were normal and showed no evidence of placental insufficiency. At 40 weeks' gestation, the patient spontaneously gave birth without any complications to a healthy boy. She was discharged with complete resolution of the skin lesions, preceded by massive desquamation of the epidermis. The 1-year follow-up of the patient revealed no relapses or new pustular eruptions.     

超级脱毛模式的临床效果观察

目的 探讨超级脱毛模式(super hair remoral,SHR)的临床效果及耐受程度.方法 采用SHR对发际、面颊、上唇、胡须、胸腹、乳晕周围、腋窝、四肢及比基尼邻近区等共进行了1 000个部位的脱毛;对一些疼痛特别敏感的部位,如上唇及头面部,治疗时采用复方利多卡因乳膏外涂1 h以上.结果 四肢、胸腹、背部及腋窝部位的毛发,一般需要4～5次治疗方可达到根治目的 ,且美容就医者无明显不适感;上唇、下颌周围毛发一般需要5～7次治疗方可收到满意效果,且需要外涂麻药,否则部分美容就医者会呈现不适感.结论 SHR模式与传统脱毛方式、光子脱毛及其他激光脱毛方式相比,具有有效性高、脱毛速度快、耐受性强等优点,值得推广.     

Familial juvenile generalized pustular psoriasis: response to methotrexate

Two siblings with generalized pustular psoriasis were admitted to the hospital. Case 1: A 7-year-old girl presented with recurrent episodes of multiple pinhead-sized pustules all over her body since the age of 3. The current episode began a week prior to the onset of the lesions. On cutaneous examination, she had generalized erythema topped with tiny pustules, scales, and crusting (Figure 1A). A Gram stain performed from the pustule and cultures taken from the pus and blood were sterile. Histopathology of the pustule was suggestive of pustular psoriasis (Figure 2). Hemoglobin was 8.8 g%. Other hematologic, microbiologic, and radiologic investigations were normal. Treatment was started with methotrexate, to which she responded dramatically with reduction in appearance of new lesions, erythema, and scaling. After 4 weeks of treatment, there was almost 90% clearance of skin lesions. Case 2: A 3-year-old boy, brother of the patient in case 1, presented with similar complaints for the past week. The episode was preceded by high-grade fever. He had generalized erythema, scaling, and edema and multiple pinhead-sized pustules, some of which were arranged in an annular configuration (Figure 1B). All investigations, as in case 1, were normal except for hypocalcemia (Ca2+ = 8.8 g%) and generalized rarefaction on x-rays of both the ankle joints. Treatment was started with prednisolone (because of poor general condition) and methotrexate. Following therapy, significant improvement was evident with reduction in erythema and cessation of appearance of new pustules. The dose of prednisolone was gradually tapered after 3 weeks and then stopped.     

超级脱毛模式的临床效果观察

目的 探讨超级脱毛模式（super hair remoral,SHR）的临床效果及耐受程度.方法 采用SHR对发际、面颊、上唇、胡须、胸腹、乳晕周围、腋窝、四肢及比基尼邻近区等共进行了1 000个部位的脱毛;对一些疼痛特别敏感的部位,如上唇及头面部,治疗时采用复方利多卡因乳膏外涂1 h以上.结果 四肢、胸腹、背部及腋窝部位的毛发,一般需要4～5次治疗方可达到根治目的 ,且美容就医者无明显不适感;上唇、下颌周围毛发一般需要5～7次治疗方可收到满意效果,且需要外涂麻药,否则部分美容就医者会呈现不适感.结论 SHR模式与传统脱毛方式、光子脱毛及其他激光脱毛方式相比,具有有效性高、脱毛速度快、耐受性强等优点,值得推广.     

超级脱毛模式的临床效果观察

目的 探讨超级脱毛模式(super hair remoral,SHR)的临床效果及耐受程度.方法 采用SHR对发际、面颊、上唇、胡须、胸腹、乳晕周围、腋窝、四肢及比基尼邻近区等共进行了1 000个部位的脱毛;对一些疼痛特别敏感的部位,如上唇及头面部,治疗时采用复方利多卡因乳膏外涂1 h以上.结果 四肢、胸腹、背部及腋窝部位的毛发,一般需要4～5次治疗方可达到根治目的 ,且美容就医者无明显不适感;上唇、下颌周围毛发一般需要5～7次治疗方可收到满意效果,且需要外涂麻药,否则部分美容就医者会呈现不适感.结论 SHR模式与传统脱毛方式、光子脱毛及其他激光脱毛方式相比,具有有效性高、脱毛速度快、耐受性强等优点,值得推广.     

Pityriasis rubra pilaris in a HIV-positive patient (Type 6 PRP)

A 45-year-old previously healthy man presented with minimally itchy spiny papular lesions of 3 years' duration and discharging nodular cystic lesions for the past 2 years. Initially, lesions appeared on his ears, followed by the gradual appearance of similar lesions over his face, back, and extremities. The lesions were not associated with photosensitivity. Over the years, the patient continued to have similar lesions without any significant response to various topical medications and oral antibiotics. There was no history of fever or any other systemic complaints and the patient denied any other significant medical problems in the past. Cutaneous examination revealed multiple, grouped, spiny papular lesions coalescing to form plaques at places over the ears, extremities, and trunk (Figure 1). Elongated, horny, follicular spires were noted on the top of the papular lesions. Multiple discharging nodular lesions with crusting were seen predominantly over the chest, abdomen, and back (Figure 2), and closed comedonal lesions were noted on the face and trunk. Multiple orange-brown scaly plaques were seen over the extensor aspect of the patient's thighs (Figure 1, inset). A few nails showed wedge-shaped thickening without subungual hyperkeratosis or other nail changes. Palms, soles, and oral mucosa were normal. Considering the atypical cutaneous findings, the clinical possibility of pityriasis rubra pilaris (human immunodeficiency virus [HIV]-associated type 6 PRP) was considered. Hematoxylin and eosin stain of skin biopsy specimens taken from the spiny papular and plaque lesions revealed marked hyperkeratosis with alternating orthokeratosis and parakeratosis with follicular keratotic plugging (Figure 3; Figure 3, inset). Irregular broad acanthosis was also detected in the epidermis. Dermis showed moderate perivascular lymphomononuclear inflammatory infiltrate. Thus, the skin biopsy was consistent with PRP. HIV serology by enzyme-linked immunosorbent assay (ELISA) with 3 different kits was found to be positive for HIV-1. The patient denied any risk factors associated with acquiring HIV infection, and laboratory values for complete blood cell count, routine serum biochemical profile, and lipid profile were normal. The patient was prescribed oral isotretinoin and asked to come for follow-up for CD4 T-cell count, but did not return.     

An unusual presentation of mucous membrane pemphigoid

A 68-year-old woman presented with recurrent mucocutaneous blisters of 6 years' duration and progressive loss of scalp hair of 2 years' duration. The disease had started as tense blisters associated with a burning sensation over the lower part of her back, gradually followed by involvement of her chest, upper part of her back, and arms. The blisters persisted for weeks before rupturing spontaneously. Four years later, she developed tense oral blisters, resulting in painful persistent erosions. At about the same time, blisters on her scalp developed, followed by erosions and hair loss. No other mucosal sites were involved. She had no symptoms of photosensitivity, joint pain, dysphagia, weight loss, or other systemic complaints. On examination, multiple tense bullae and well-defined deep erosions were seen over the hard and soft palates (Figure 1). Cutaneous examination revealed a few tense bullae on normal-looking skin over her abdomen (Figure 2 inset) and arms and areas of scarring at the site of healed lesions on her back. Her scalp had bullae of similar morphology, crusted erosions, and cicatricial alopecia at the site of previous lesions involving a large area of scalp (Figure 2). Examination of the other mucosae did not reveal any abnormality. Histologic studies with hematoxylin and eosin stain of a skin biopsy specimen revealed deroofed subepidermal bullae with dense dermal inflammatory infiltrate predominantly composed of eosinophils (Figure 3). Direct immunofluorescence of a biopsy specimen from perilesional skin revealed linear deposits of immunoglobulins M and G and of C3 at the dermoepidermal junction which was consistent with mucous membrane pemphigoid. This patient was prescribed prednisolone 30 mg daily and dapsone 100 mg daily, following which there were no new blisters. At 3 months' follow-up, previous erosions had partially healed.     

Folliculocentric lichen sclerosus et atrophicus

A 71-year-old black woman presented to the dermatology clinic with a several-year history of increasing numbers of itchy white spots spreading over the chest, back, elbows, and legs. In 2004, the patient developed clusters of mildly pruritic hypopigmented and depigmented macules on her back and left shoulder along with depigmented flat papules on the lower extremities. The patient's condition was unresponsive to topical triamcinolone and tacrolimus, after which she was lost to follow-up for 3 years until her entire chest became involved. Her medical history was significant for hypertension and diabetes mellitus and her medications included amlodipine, aspirin, atorvastatin, hydrochlorothiazide, irbesartan, and metformin. Family history, social history, and review of systems were unremarkable. Physical examination revealed multiple shiny white perifollicular macules, some with slight scale, on the trunk (Figure 1) and extremities (Figure 2). On the left shoulder and back were 2 shiny plaques with hypopigmented macules and follicular scale within. Punch biopsies from the abdomen and back showed atrophy of the epidermis, edema and sclerosis of the papillary dermis, and a patchy lichenoid lymphocytic infiltrate (Figure 3). Despite inspecting multiple step sections, we were unable to histopathologically confirm the clinically apparent folliculocentric distribution. Based on the clinical and histological findings, extragenital lichen sclerosus et atrophicus (LSA) was diagnosed and the patient was treated with clobetasol proprionate 0.05% ointment. While being prepared for narrowband UV-B therapy, a serologic screen revealed antinuclear antibodies at 1:640 with a homogeneous pattern. A subsequent rheumatologic evaluation was negative for autoimmune disorders; pertinent negative serologies included anti-double-stranded DNA, smooth muscle, mitochondrial, thyroid, and parietal cell antibodies.     

Subepidermal calcified nodule in a 7-month-old boy

A 7-month-old healthy white boy presented for evaluation of a papule on his right ear. His mother reported a small cut-like lesion at this site shortly after birth that had gradually grown over time. The patient was born full term via repeat cesarean section. On physical examination, located on the right superior helix, there was a 5-6 mm, pink, firm papule with a rough scaly surface and peripheral erythema (Figure 1). The lesion was shave excised and sent for histologic analysis. Microscopic examination revealed a defect of the epidermis with underlying fibrin deposits (Figure 2). Subjacent to the ulcer, there was chondroid tissue, granulation tissue with prominent vascularity, and an extensive amount of amorphous calcified material throughout the dermis extending to the base of the specimen (Figure 3). The clinical and pathologic findings were consistent with a subepidermal calcified nodule (SCN), an uncommon idiopathic calcinosis. The lesion was surgically removed with good results.