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Open Access Publications from the University of California

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Dermatology Online Journal is an open-access, refereed publication intended to meet reference and education needs of the international dermatology community since 1995. Dermatology Online Journal is supported by the Department of Dermatology UC Davis, and by the Northern California Veterans Administration.

Volume 21, Issue 9, 2015

Case Report

Generalized linear IgA dermatosis with palmar involvement

Linear IgA bullous dermatosis (LABD) is a sub-epidermal blistering disorder characterized by deposition of IgA along the basement membrane zone (BMZ) as detected by immunofluorescence microscopy. The diagnosis is made by clinicopathologic correlation with immunofluorescence confirmation. Differentiation from other bullous dermatoses is important because therapeutic measures differ. Prompt initiation of the appropriate therapies can have a major impact on outcomes. We present three cases with prominent palmar involvement to alert the clinician of this potential physical exam finding and to consider LABD in the right context.

Opioid associated intravenous and cutaneous microvascular drug abuse (skin-popping) masquerading as Degos disease (malignant atrophic papulosis) with multiorgan involvement

Background: In 2012, a nephrologist reported the development of a multiorgan thrombotic syndromic complex resembling thrombotic thrombocytopenic purpura (TTP) in patients who were abusing long acting oxymorphone hydrochloride; all patients had hemolytic anemia and thrombocytopenia.

Objective: Herein, we report another case involving a 31-year-old woman who self intravenously administered dissolved oral oxymorphone resulting in thrombotic sequelae  resembling Degos disease.

Methods: Formalin-fixed and paraffin embedded skin biopsies were prepared according to standard protocols for H&E and immunohistochemistry.

Results: The clinical presentation and biopsy findings were held to be indicative of  Degos disease/malignant atrophic papulosis (MAP) but with unusual clinical features including renal failure and severe respiratory insufficiency. Given the efficacy of eculizumab in the treatment of the acute thrombotic phase of Degos disease/MAP, the patient received this drug, resulting in rapid resolution of signs and symptoms associated with her multiorgan failure. Although she developed recurrent cutaneous ulcers despite complete complement inhibition with eculizumab., her other extracutaneous manifestations did not recur. The patient’s pre and post eculizuamb skin biopsies showed a striking pauci-inflammatory thrombogenic vasculopathy associated with marked endothelial cell injury along with deposits of C3d and C4d within the cutaneous vasculature; the C5b-9 deposits were limited to the pre-eculizumab biopsy. We discovered that her syndromic complex was a self-inflicted one related to the localized administration of dissolved oxymorphone.

Conclusion: Our patient’s biopsy along with the rapid response to eculizumab indicates that this distinct thrombotic microangiopathy is another complement mediated thrombotic microangiopathy syndrome. Opioid thrombotic microangiopathy has a varied clinical presentation and can mimic other catastrophic microangiopathy syndromes, all of which have in common a responsiveness to complement inhibition.

Case Presentation

A 7-year-old with indurated skin and unilateral progressive joint immobility: A case of stiff skin syndrome

Stiff skin syndrome is a rare sclerotic condition that presents during infancy or early childhood. It has an insidious chronic course and may lead to significant co-morbidity and reduced quality of life. Often, affected individuals experience impaired ambulation and immobilization related to joint involvement. Clinically, it may resemble other sclerotic diseases, so histopathological evaluation is necessary to establish a diagnosis. As it is a condition with limited treatment options, prompt diagnosis and early initiation of physical therapy is crucial to prevent joint restriction and maintain quality of life. We describe a case of a 7-year-old with stiff skin syndrome, and review the literature to discuss the clinical presentation, histological findings, and management of this condition.

Sporotrichoid granuloma annulare-like dermatitis associated with systemic B-cell lymphoma

Importance: Granuloma annulare is typically a benign, self-limited disease. Atypical presentations have been reported in association with systemic disease, including malignancy. Such patients may require additional diagnostic studies to assess for underlying malignancy. We report a patient with extensive sporotrichoid granuloma annulare-like dermatitis in association with systemic B-cell lymphoma.

Observations: An 83-year-old man with a three-year history of progressive sporotrichoid annular plaques and nodules on the arm developed ipsilateral retroauricular palpable lymphadenopathy, the latter consistent with B-cell lymphoma. Multiple skin biopsies of the plaques and nodules revealed granuloma annulare-like dermatitis. Lesions were unresponsive to intralesional and intramuscular corticosteroids, antibiotics, and antifungal agents, but rapidly improved following initiation of rituximab to treat his underlying lymphoma.

Conclusions and Relevance: Atypical presentations of granuloma annulare including granuloma annulare-like dermatitis warrant evaluation for systemic malignancy in a subset of patients.

Scleromyxedema secondary to hepatitis c virus and successfully treated with antiviral therapy

Scleromyxedema (SM) is a chronic and progressive fibromucinous disease with no known etiology. We report a patient with scleromyxedema associated with hepatitis C virus, successfully treated with interferon and ribavirin therapy.

Phacomatosis cesiomarmorata with hypospadias and phacomatosis cesioflammea with Sturge-Weber syndrome, Klippel-Trenaunay syndrome and aplasia of veins -- case reports with rare associations

Phacomatosis pigmentovascularis (PPV) is a rare genodermatosis characterized by the co-existence of an extensive vascular and a pigmentary nevus with or without extracutaneous manifestations. We report two such rare cases. The first is a 3-year-old boy exhibiting a rare association of cutis marmorata telangiectatica congenita with aberrant dermal melanocytosis along with hypospadias and melanosis oculi (traditionally classified as PPV type Vb or phacomatosis cesiomarmorata - Happle’s classification). The other patient is a 5-year-old boy with Sturge-Weber syndrome, Klippel-Trenaunay syndrome, aplasia of iliac, femoral, and popliteal veins and congenital heart disease, associated with aberrant dermal melanocytosis and melanosis oculi (also classified as PPV type IIb or phacomatosis cesioflammea). These sporadic cases display a unique constellation of additional, previously unreported systemic associations, which will further expand the clinical spectrum of phacomatosis pigmentovascularis.

Eruptive milia and comedones during treatment with dovitinib

Dovitinib (TKI258) is a multi-targeted receptor tyrosine kinase inhibitor currently under clinical trials for a wide variety of cancers. Well-known side effects include nausea, vomiting, diarrhea, and fatigue. To date, there have only been only two reported cases with skin manifestations as a side effect. We report a case of eruptive facial milia and comedones in the setting of dovitinib treatment for metastatic gastrointestinal cancer. This case is unique as the clinical presentation was more rapid in onset and showed an absence of inflammatory lesions. Although the pathogenesis for skin manifestations is presently unknown, we present this case to increase awareness of potentially under-reported cutaneous side effects.

Trichophytum rubrum endonyx onychomycosis resistant to standard oral and topical therapies

We present a 45 year-old man with an eight-year history of discoloration of the nail plate on his left hallux. He had been treated with two courses of oral terbinafine and topical 8% ciclopirox for presumed onychomycosis. On exam, his left great toenail contained a wide yellow-white longitudinal band involving a majority of the nail plate. No subungual debris, hyperkeratosis, or paronychial inflammation was present in the affected nail. Histopathology of the nail plate revealed numerous fungal elements arranged transversely and longitudinally, solely within the keratin layers of the nail plate; these were highlighted with periodic acid-Schiff (PAS) stain confirming endonyx onychomycosis. Cultures grew Trichophyton rubrum. All types of onychomycosis under the new classification system proposed by Hay et al. have now been associated with T. rubrum. Endonyx related to T. rubrum may be a particularly difficult infection to treat with oral or topical agents owing to the absence of robust local immune response and limited drug penetration to the interior nail plate. Physicians should be aware that this type of infection may require treatment with dual-agent therapy or alternative modalities including chemical or surgical plate avulsion or photodynamic therapy.

Generalized lentiginosis in an 11 year old boy

Generalized lentiginosis refers to generalized lentigines without systemic abnormalities, characterized by multiple brown or black macules owing to increased proliferation of melanocytes. There are also lentiginosis syndromes associated with systemic abnormalities such as Peutz-Jeghers syndrome, Leopard syndrome, and Carney complex. Generalized lentiginosis can be diagnosis by patient’s history, physical and laboratory examination, and histopathology. We report an 11-year-old boy who presented with multiple dark brown macules, varying in size, but less than 0.5 cm, with no abnormalities of other systemic organs.

Bullous pilomatricoma: a rarely reported variant of pilomatricoma

Pilomatricomas are cutaneous adnexal tumors with matrical differentiation. We report and describe a rare variant called bullous pilomatricoma.

Two sisters with Reed’s syndrome: treatment with pregabalin

Cutaneous leiomyomas, which originate in the arrector pili muscles of the skin are rarely seen benign cutaneous tumors. Sometimes familial cutaneous and uterine leiomyomatosis can occur together, an autosomal dominant genetic condition called Reed syndrome or familial leiomyomatosis cutis et uteri. This disorder can be accompanied by malignancies, particularly by renal carcinoma. In this paper, two sisters with Reed syndrome are presented in view of the rarity of the disorder and good response to pregabalin therapy.

Photo Vignette

Localized and linear lichen planopilaris over the face and scalp with associated alopecia – clinical and dermoscopy pattern

A case of localized lichen planopilaris over the face, extending to the scalp is presented.This case highlights the different variations, which can occur in the presentation of localized lichen planus. The case was unique because of the extension of the lesions into the scalp. We also wanted to demonstrate the peculiar dermoscopic patterns of active lichen planopilaris when occurring over the face.

What is the prognostic significance of acrometastases?

In contrast with bone metastasis, acrometastases are uncommon and are associated with advanced cancer. We report the cases of two patients with atypical lesions of the fingers in a context of cancer, in which biopsies confirmed a metastasis. Patients died rapidly before treatment was initiated. We discuss the characteristics of these atypical metastatic sites, associated with a generally poor prognosis.

Immediate resolution of severe bullous chronic regional pain syndrome with onset of spinal paralysis

Complex regional pain syndrome (CRPS) is an incompletely understood disorder characterized by progressive regional pain and sensory changes, with fluctuating cutaneous edema and erythema. We describe a patient with a rarely reported severe bullous CRPS variant on the left lower extremity, which resolved immediately upon developing spinal paralysis.

Commentary

Even well-controlled psoriasis patients have unmet treatment needs regardless of disease severity

Background: Though psoriasis is chronic and recurring, current options can help many patients achieve good disease control.New treatments could provide greater improvement in objective disease, but it is not clear that there is room for improving subjective outcomes, particularly for patients who are already “well-controlled.”Objective: To analyze treatment satisfaction of well-controlled patients with psoriasis in two patient populations of very different disease severity.Methods: Patients with well-controlled psoriasis on topicals (mild psoriasis) only or on biologics (moderate-to-severe psoriasis) were queried by telephone about perceptions of disease control, improvement level, overall treatment satisfaction, and consideration for trying new treatments. Satisfaction scores and feedback were analyzed to assess treatment type and satisfaction level associations.Results: The majority of patients, regardless of disease severity, would consider trying new treatments for psoriasis, though patients with mild psoriasis were less satisfied with their disease control, level of improvement, and overall treatment. Patient feedback revealed widespread treatment dissatisfaction, non-adherence, and inconvenience.Limitations: The patient population was from one university setting.Conclusion: 16% of patients with psoriasis meet study definition for “well-controlled” disease. Less than 20% of these well- controlled patients with psoriasis are satisfied with their current level of control and clearance and would not consider trying new treatments, suggesting the majority of well-controlled patients, regardless of disease severity, still have unmet needs. Strongerpatient-physician communication may contribute to patient-based, comprehensive care in concordance with a health system that isheaded towards financial incentives for better patient satisfaction.

Additional evidence that rosacea pathogenesis may involve demodex: new information from the topical efficacy of ivermectin and praziquantel

Additional evidence that Demodex folliculorum may contribute to the pathogenesis of papulopustular rosacea are new studies of two topical antiparasitic agents. Ivermectin and praziquantel have recently been shown to be effective in decreasing the severity of papulopustular rosacea. These two agents significantly differ in molecular structure, but yield similar antiparasitic mechanisms of action. Higher numbers of Demodex mites are found in the skin of patients with rosacea than in people with normal skin. If Demodex play a role in pathogenesis, then hypersensitivity to the mites, their flora, or their products could explain the observed efficacy of antidemodectic therapy.

Letter

Generalized pruritus in dysmetabolic hyperferritinemia treated by phlebotomy

This paper describes a case of pruritus caused by dysmetabolic hyperferritinemia treated by multiple phlebotomies.A 63-year-old man was followed for generalized pruritus, which was resistant to the usual treatments. He presented with metabolic syndrome. Physical examination showed only excoriations and lichenification on the skin. The serum ferritin was high at 1043 ng/ml, with transferrin saturation at 67%. The other biological investigations and genetic tests for hemochromatosis were negative.In spite of the dietary measures, the ferritin level was still high (853ng/ml). Magnetic resonance imaging confirmed hepatic iron overload.The association of hyperferritinemia, hepatic iron overload, and metabolic syndrome led to the diagnosis of dysmetabolic hyperferritinemia.Phlebotomies are an unusual treatment, but because the pruritus and hyperferritinemia were still present, phlebotomy wasinitiated. After 19 months, the patient reported improvement of his pruritus and normalization of ferritin levels.