Pityriasis rubra pilaris

Pityriasis rubra pilaris (PRP) is a rare papulosquamous disease with typical onset during the first and fifth decades. The skin disorder normally starts on the scalp and spreads caudally within a few weeks. It often results in a generalized erythroderma with sharply demarcated islands of sparing ("nappes claires"). A 65-year-old patient with severe PRP showed good clinial improvement after 8 months of treatment when treated with acitretin in combination with phototherapy and systemic gluocorticosteroids.     

Topical Retinoic Acid in Bullous Ichthyosiform Erythroderma

A five-year-old patient with bullous ichthyosiform erythroderma is being treated with topical retinoic acid with improvement in her condition. She is now able to attend school and take her place in the community. This may be the first published account of the use of this substance in the bullous form of the condition.     

THE USE OF RETINOIC ACID IN CONGENITAL ICHTHYOSIFORM ERYTHRODERMA *

SUMMARY. . A patient with congenital ichthyosiform erythroderma was treated with retinoic acid, topically and orally. By both methods a considerable improvement in the condition was achieved. The background to the use of this agent and some hypotheses as to how it may influence keratin are discussed.     

Erfolgreiche Behandlung einer Pityriasis rubra pilaris (Typ 1) durch Kombinations-Therapie mit Infliximab und Methotrexat

Pityriasis rubra pilaris (PRP) is often difficult to treat. A 65‐year‐old women presented with a two week history of widespread erythroderma and scaling with areas of sparing (nappes claires). She also had follicular hyperkeratoses and palmar fissuring. The clinical picture and histology led to the diagnosis of PRP. She failed to respond to initial therapy which included topical and systemic corticosteroids. She was then treated with intravenous methotrexate (MTX) 15–30 mg weekly. Because of the poor response we intensified her regime with infliximab (5 mg/kg). Altogether our patient received infliximab three times together with MTX, which was later given orally. We report for the first time the successful combination of infliximab and MTX for the treatment of PRP.     

Generalized pustular psoriasis responsive to PUVA and oral cyclosporin therapy

A patient with acute generalized pustular psoriasis was successfully treated with a combination of oral cyclosporin (6 mg/kg per day) and photochemotherapy (PUVA). Although early inpatient treatment with weak topical steroids and PUVA produced initial improvement, the patient's clinical condition fluctuated, with the subsequent development of erythroderma. The addition of oral cyclosporin produced dramatic improvement within 1 week of its commencement. The patient remained in remission 12 months following cessation of therapy.     

Dapsone-induced erythroderma with Beau's lines

A 35-year-old female with borderline lepromatous (BL) leprosy who suffered from dapsone-induced erythroderma is reported. Sudden onset of erythroderma gave rise to a temporary arrest of the function of nail matrix with the resultant Beau's lines. She rapidly recovered with omission of dapsone and therapy with systemic corticosteroids and a topical emollient. In view of the potentially fatal hypersensitivity reaction, we suggest that any patient on multidrug therapy for leprosy needs an urgent referral to a dermatologist if the patient develops a skin rash during the first two months of treatment.     

Four cases of sebopsoriasis or seborrheic dermatitis of the face and scalp successfully treated with 1a-24 (R)-dihydroxycholecalciferol (tacalcitol) cream

A 71-year-old woman visited our clinic due to the presence of widespread scaly erythema on her face, scalp, and lower extremities. She was tentatively diagnosed as having seborrheic dermatitis but the symptoms were difficult to distinguish from psoriasis vulgaris. As a result, she was diagnosed as having sebopsoriasis. She was treated topically with an active vitamin D3 compound, 1a-24 (R)-dihydroxycholecalciferol D3 (tacalcitol) cream. She applied tacalcitol cream twice daily for 4 weeks, and her facial eruptions thus cleared up completely. No recurrence was observed for 2 months thereafter, even though the use of tacalcitol cream was stopped. To investigate whether or not tacalcitol cream is generally effective for the treatment of such seborrheic dermatitis-like eruptions, three more patients were treated with tacalcitol cream. All patients exhibited scaly erythematous macules on the face and/or scalp, and their eruptions improved rapidly with tacalcitol cream. Tacalcitol cream was thus found to be effective and useful for the treatment of both sebopsoriasis and even seborrheic dermatitis of the face and scalp.     

Muir-Torre syndrome: a case of this uncommon entity

A 69-year-old Hispanic woman presented for the evaluation of nodules on the head and back. In the past, she had been treated for basal cell carcinoma (BCC) of the face; the referring physician was concerned that the new lesions might also be BCC. The patient had an extensive past medical history. In addition to BCC, she had been treated for breast cancer, colon cancer, and cervical cancer prior to emigrating to the USA. Her colonic malignancy had been localized proximal to the splenic flexure. She also had a history of colonic polyps and distal colonic villous adenoma. She denied ever being treated with radiation. Further details of her medical history and cancer staging were not available. Her family history was significant for a sister with colon cancer and transitional cell carcinoma of the urinary bladder. In addition, she had a great aunt with oral cancer and a great uncle with lung cancer. Neither the patient or her relatives had any history of tobacco use. On physical examination, in addition to scars from a radical mastectomy and midline abdominal laparotomy, four skin lesions were noted: two on the scalp, one on the tragus, and one on the mid-back. The first lesion on the vertex of the scalp was a yellow-brown waxy papule measuring 0.6 x 0.5 cm. This lesion was similar to that on the mid-back, except in size. The lesion on the back measured 1.2 x 1.0 cm. The second lesion on the frontal scalp measured 0.8 x 0.6 cm and was red-brown with a pearly appearance and some central hyperkeratosis. The tragus lesion was similar in appearance to that on the frontal scalp. Shave biopsies of all lesions were obtained. The lesions on the scalp and mid-back revealed lobules of sebaceous cells in the dermis with a minority of surrounding basaloid cells, consistent with a diagnosis of sebaceous adenoma (Fig. 1). Although the lesion on the frontal scalp also showed sebaceous differentiation, there were a greater number of basaloid cells, some with hyperchromatic nuclei and mitotic figures; this was consistent with a diagnosis of sebaceous epithelioma (Fig. 2). The final lesion (tragus) was histologically consistent with a keratotic BCC. No further treatment was required for these benign sebaceous tumors, but their presence defined our patient's condition as Muir-Torre syndrome. Mohs' micrographic surgery was performed on the tragus BCC and the margins were tumor free in one stage. The patient returned 1 year later with a lesion anterior to the left axilla which was biopsied to rule out BCC (Fig. 3). Histologically, this lesion was also consistent with sebaceous epithelioma.     

Ichthyosis bullosa of Siemens responds well to low-dosage oral retinoids

Two patients with ichthyosis bullosa of Siemens (IBS) and one patient with bullous ichthyosiform erythroderma of Brocq (BIE) were treated with etretinate. Two additional patients with IBS received acitretin. All the patients had a marked improvement when on retinoids and the maintenance dose required was for IBS 10-25 mg per day. The patient with BIE was on a maintenance dose of 40-60 mg per day.     

Cyclosporin Treatment Improves Skin Findings in Omenn Syndrome

Omenn syndrome is a combined immunodeficiency characterized by a generalized erythematous skin rash, enlarged lymph nodes, hepatosplenomegaly, severe susceptibility to infections, eosinophilia, and hyperimmunoglobulinemia E. A 3‐month‐old girl was admitted to our hospital with a history of recurrent sepsis. Physical examination revealed severe erythroderma, hepatosplenomegaly, lymphadenopathy, and failure to thrive. Laboratory findings revealed leukocytosis, lymphocytosis with high CD3 T‐cells, a high CD4:CD8 ratio, absence of CD19 B‐cells, high eosinophil count, and low immunoglobulin levels. A heterozygote RAG1 gene mutation was found. She had itchy, scaling, ichthyosiform erythroderma and protracted diarrhea. Cyclosporin treatment up to 10 mg/kg effectively resolved erythroderma and lowered total eosinophil counts, and she gained weight during treatment. Since extensive erythroderma with generalized itching causes patient discomfort in Omenn syndrome, cyclosporin treatment can be considered while waiting for treatment with hematopoietic stem cell transplantation.     

Neonatal dermatophytosis: report of a case and review of the literature

Tinea capitis is a common fungal infection in children but rare among neonates. We present a case of a 3-week-old infant with multiple scalp lesions for 1 week. A diagnosis of tinea capitis due to Microsporum canis was made based on clinical morphology, Wood's lamp, and biopsy with a positive PAS stain. She was effectively treated with oral griseofulvin and ketoconazole shampoo.     

Generalized erythrodermic pemphigus foliaceus in a child and its successful response to rituximab treatment

Pemphigus foliaceus is an autoimmune disease that clinically manifests with cutaneous blisters of the superficial skin. The nonendemic or sporadic form of this entity is rare in children and typically presents with a milder, more localized rash that usually follows a benign course of short duration. We describe an affected patient atypical in both her young age and the severity of skin findings. Our patient presented with a full body exfoliative erythroderma at 21 months of age. After an extensive work-up to determine the etiology of her exfoliative erythroderma, direct and indirect immunofluorescence studies confirmed the diagnosis of pemphigus foliaceus. Rituximab therapy was initiated based on the patient's refractory disease course to multiple immunosuppressive agents. Rituximab is a therapeutic monoclonal antibody targeting CD20, an integral membrane protein highly expressed on the surface of pre-B lymphocytes and activated mature B lymphocytes. The patient's skin exhibited marked clinical improvement after the start of rituximab infusions over 12 weeks. Her initial desmoglein 1 antibody level was greater than 1:1280, which decreased to 1:16 after seven rituximab treatments. She has had no skin flares since initiating treatment with rituximab therapy. Based on this clinical and serologic response, the use of rituximab may be helpful in the treatment of pediatric pemphigus foliaceus refractory to mainstays of therapy.     

PLICA NEUROPATHICA AFTER USING HERBAL SOAP

A 22-year-old woman was referred to dermatology from oncology for an opinion regarding the sudden matting of scalp hair of 10 days duration. A case of primary infertility for 7 years, this patient was undergoing chemotherapy with doxorubicin, cyclophosphamide, and cisplatin after operative removal of a papillary cystadenoma (clear ceil type) from her left ovary 40 days previously.
She had noticed a sudden matting of her hair 3 to 4 hours after using a soap containing Acacia concinna for washing her hair. All efforts to disentangle the matted hairs were futile. She was feeling very uncomfortable because of the heavy solid mass of tangled hair. She told of rubbing her wet hair and using a wet towel to cover her hair after use of the soap. She denied earlier use of the soap, shampoo, setting lotions, bleaches, or hair dye. She had not cut her hair since early childhood.
Examination showed a huge, firm almost stony mass of severely tangled hair on her scalp, which was more marked over the vertex and occipital areas (Figs. 1 and 2). The hairs in the frontal area were free, thin, and straight. The skin over the entire scalp was normal. On light microscopy, a few hairs showed features of trichorrhexis nodosa. Routine hematology was normal. The patient was unable to tolerate the pain and discomfort because of the stony hard mass. She shaved her head and offered her hair in a temple, as is a common custom in parts of South India.     

Broad-band UVB fiber-optic comb for the treatment of scalp psoriasis: a pilot study

Abstract Background Scalp psoriasis is a frustrating condition with limited treatment options. Our objective was to test a new fiber-optic broad-band UVB comb device for the treatment of this condition. Methods Scalp psoriasis on adult subjects was treated three times a week for up to 12 weeks with a fiber-optic UVB comb. A minimum 50-cm(2) scalp area served as the control. Starting doses were based on the standard minimal erythema dose (MED) with increments up to 20% for subsequent treatments. Results Fourteen subjects completed the study without sunburn reactions or blisters. Treated sites showed a mean improvement in the modified PASI score of 3.6. The difference in the mean modified PASI scores between the control and treated sites was 3.9 (< 0.0001). Conclusions This fiber-optic broad-band UVB comb can safely and effectively treat scalp psoriasis.     

Omenn's syndrome: lessons from a red baby

Exfoliative dermatitis and erythroderma in infancy are rare. Clinicians need to be alert to the possible diagnosis of Omenn's syndrome (OS), a rare form of combined immunodeficiency in infants presenting with exfoliative dermatitis, erythroderma, recurrent infections, eosinophilia and raised IgE. OS is fatal unless treated by bone-marrow transplantation (BMT). We describe a 3-week-old girl who presented with a widespread scaly erythematous rash and stomatitis, and was initially treated for presumed atopic eczema and primary herpes stomatitis. Aged 3 months, she developed erythroderma, diarrhoea and hepatosplenomegaly associated with eosinophilia, raised serum IgE and low IgG, IgA and IgM levels, abnormal lymphocyte populations and skin histology, consistent with a diagnosis of OS. She remains well 16 months after a human leucocyte antigen-matched bone-marrow transplant from an unrelated donor.     

Epidermolytic variant of hereditary palmoplantar keratoderma

The seventh family with autosomal dominant epidermolytic palmoplantar keratoderma is reported. The lesions are clinically indistinguishable from Unna-Thost disease but resemble epidermolytic hyperkeratosis (bullous ichthyosiform erythroderma) histopathologically. A skin biopsy is essential for making the correct diagnosis. One of our patients was treated with isotretinoin for 13 weeks without significant improvement.     

Temporal arteritis presenting with scalp ulceration

We report the case of a 75-year-old-woman who presented with bilateral scalp ulcerations and blindness, accompanied by severe headache and scalp tenderness, due to bilateral temporal arteritis without systemic involvement. A biopsy taken from the border of an ulceration showed evidence of giant cell arteritis. She was treated with oral prednisone, 60 mg per day. The ulcerations healed in a few weeks but the vision loss was irreversible. This case highlights for temporal arteritis the importance of accurate and timely diagnosis as well as the need for prompt therapy with systemic steroids in order to avoid major complications, namely loss of vision. It also demonstrates that scalp necrosis and ulcerations are skin signs associated with a poor prognosis.     

Circumscribed alopecia: an unusual manifestation of pediculosis capitis

A healthy 10-year-old girl was referred for evaluation of patchy hair loss on the scalp of longer than 6 months duration. She had been diagnosed and treated for head lice approximately 1 month before onset of the hair loss. Examination of the scalp showed discrete ovoid patches of hair loss at the vertex scalp. A scrape of the area of hair loss was performed, and a nit was visible on microscopic examination. Focal hair loss may represent an atypical manifestation of ongoing pediculosis capitis.     

Erythroderma,Hair Loss,and Sinus Histiocytosis with Massive Lymphadenopathy in a XYY-Male

This paper presents a case of sinus histiocytosis with massive lymphadenopathy (SHML) presenting as erythroderma in an XYY-male aged 27. The patient had little or no scalp, axillary, or pubic hair. Histologically, lymph nodes indicated dilatation of the sinuses with proliferation of benign-appearing histiocytes possessing abundant clear cytoplasm, admixed with giant cells and pericapsular fibrosis. Infiltrating histiocytes in the lymph nodes were OKM-1 (CD11b) positive, which is characteristic of monocyte-granulocyte lineages. In the present case, the abnormal presence of erythroderma, hair loss, SHML, and a chromosomal abnormality, XYY, were thought to be incidental features.     

Nonsynchronized segmented heterochromia in black scalp hair

Nonsynchronized segmented heterochromia in black scalp hair is a rarely reported entity, the only previous report being described in association with iron deficiency anaemia. A 14-year-old girl presented with a 2-year history of nonsynchronized segmented heterochromia. She was otherwise well and her serum iron, copper, zinc and protein levels were all within the normal range. She had no clinical evidence of vitiligo or alopecia areata. This patient is believed to represent the first reported case of nonsynchronized segmented heterochromia in black scalp hair as a presentation of premature greying of the hair.