Penile pemphigus

BACKGROUND: Penile skin involvement in patients with pemphigus vulgaris has been rarely reported. This study describes the involvement of penile skin in 12 patients with pemphigus vulgaris. OBSERVATIONS: Of the 12 patients, 10 had involvement of the skin and mucous membranes. Two patients had involvement of the oral mucosa only and no cutaneous involvement. None of the patients had urethral involvement. We did not observe isolated involvement of penile skin only, in the absence of disease elsewhere. Using monkey esophagus as substrate, all the patients had detectable levels of antibodies to keratinocyte cell surface antigen(s) in their serum samples. Since histological, serological, and clinical evidence of pemphigus was present, biopsies of the penile skin were not done. Topical therapy was concomitantly used with systemic therapy. Once treated and resolved, recurrence of penile lesions was not observed during the long-term follow-up. CONCLUSIONS: Involvement of penile skin is rare and was observed with the presence of pemphigus lesions in other areas of the body. Lesions involving the penile skin were most commonly seen on the glans. No sequelae or functional abnormalities were observed on long-term follow-up.     

Necrotizing sarcoid granulomatosis with skin and pulmonary involvement

We present a rare case of necrotizing sarcoid granulomatosis (NSG) with skin and pulmonary involvement. NSG with extrapulmonary involvement occurs infrequently, and reports involving skin manifestations in NSG are even more rare.     

Diffuse large B cell lymphoma progression with skin involvement: A case report

Diffuse large B cell lymphoma (DLBCL) constitutes the most frequent subtype of all non-Hodgkin's lymphomas. DLBCL is an aggressive disease and extranodal involvement is seen in approximately 30% of patients and most common extranodal sites are gastointestinal tract and skin. Skin involvement may be either primary or secondary. Secondary cutaneous lymphoma has a worse prognosis. The case is here reported of a 56-year old male DLBCL patient with cutaneous lesions and aggressive clinical course. The patient had no skin lesions at diagnosis and during follow up and treatment period, skin, cerebrospinal fluid and bone marrow involvement was occurred. Salvage chemotherapy and autologous stem cell transplantation was planned but the patient died before the second cycle of salvage chemotherapy. In contrast to primary cutaneous lymphoma, which tends to be more indolent, secondary skin involvement is associated with unfavourable prognosis. In conclusion it should be kept in mind that skin can be involved in lymphoma patients and in these cases, skin biopsy should be performed rapidly.     

Osteosarcoma of the skin

Background Osteosarcoma is a common malignancy, although skin metastasis is rare. We sought to review the incidence, epidemiology, risk factors, and prognosis of osteosarcoma with skin involvement. Methods We conducted a retrospective chart review that covered 30 years and involved clinic patients younger than 18 years who had cutaneous metastases of osteosarcoma. The main outcome measure was histologic documentation of both primary tumor and metastatic lesion in the skin. Results Two patients were found to have osteosarcoma with skin involvement. No unifying factors were identified. Conclusions Although osteosarcoma is a common malignancy with frequent metastases, involvement of the skin is rare. Further studies to identify risk factors and subsequent prognosis are necessary. Nevertheless, unidentifiable skin lesions in a patient with a history of osteosarcoma should be investigated to rule out tumor metastasis, regardless of cancer status.     

Biomechanical measurement of skin distensibility in scleredema of Buschke associated with multiple myeloma

We report a case of scleredema of Buschke associated with IgG kappa monoclonal hypergammaglobulinaemia. After myeloma polychemotherapy an improvement in skin involvement was observed and confirmed by means of noninvasive skin elasticity measurements. This suggests a relationship between the two diseases. The bioengineering method used can be useful for early detection and monitoring the skin involvement in patients with this disease association.     

Cutaneous involvement in Hodgkin's lymphoma: report of two cases

Hodgkin's lymphoma (HL) comprises 20 to 30% of all lymphomas. Skin involvement is almost always secondary to visceral or nodal involvement. Secondary cutaneous HL is rare, occurring in only 0.5 to 3.4% of the cases. Herein we report two cases of skin involvement in Hodgkin's disease. One was a 25-year-old female admitted with a draining sinus in the neck. The other was a 19-year-old female admitted with a neck mass and skin papules on her chest and arm. Skin involvement may antecede or can be seen during the course of HL. Better understanding of such cutaneous involvement, which occasionally may be the initial sign of HL, is needed, and a biopsy of any suspicious skin lesion should be considered.     

Shwachman Syndrome: A Case Report

Cutaneous involvement is frequent in Shwachman syndrome, and includes various degrees of dry skin, and eczematous and ichthyosiform lesions. A 12-year-old boy with Shwachman syndrome had cutaneous involvement characterized by dry skin, perioral dermatitis, and follicular keratosis. Polymorphonuclear motility was decreased. A nutrition work-up showed a decrease in liposoluble vitamins, and suggested a causative link with the skin lesions.     

Skin involvement as the presenting sign of internal carcinoma. A retrospective study of 7316 cancer patients

From tumor registry data of 7316 cancer patients, we found 367 cases (5.0%) with skin involvement. Skin involvement was present at the time of presentation in 92 patients (1.3%), only 26 of whom had remote metastases. Skin involvement was the first sign of cancer in 59 patients (0.8%); 22 had direct extension of their tumor into the skin, 20 had local metastases, and 17 had distal metastases. Direct invasion was most common with breast cancer and second most common with oral cavity cancer. Local metastases were also most frequently caused by breast cancer but occurred in surgical scars in three women with pelvic cancer and in perianal abscesses in one patient with rectal carcinoma as well. Except for metastases from unknown primary sites, distant metastases were rare as presenting signs, and their origins were widely distributed. Our data show that internal cancer uncommonly presents with skin involvement. Nevertheless, an index of suspicion should be maintained and biopsy performed, particularly for nonhealing ulcers, persistent indurated erythema, and unexplained skin nodules.     

The correlation between cutaneous IgM deposition and renal involvement in adult patients with Henoch-Schönlein purpura

Background

Henoch-Schönlein purpura (HSP) is a systemic and immune complex-related leukocytoclastic vasculitis. A positive correlation has been found between IgM deposition along with IgA in skin lesions diagnosed by direct immunofluorescence (DIF) and renal involvement with HSP.

Objectives

We sought to investigate the relationship between the systemic associations of the disease and the severity of the skin lesions, as well as the immune deposition findings of the lesional skin provided by DIF in patients with HSP.

Materials & Methods

Between 2001 and 2012, 47 patients with HSP were investigated at our dermatology clinic. Epidemiologic data, laboratory findings, localizations of the palpable purpura, existence of bulla or necrosis in the skin lesions, accompanying systemic involvement, and DIF findings of skin biopsy specimens were recorded.

Results

Of the 47 patients 22 were men and 25 were women (age range 16–88 years, mean 42.2). Among all cases with skin involvement, we found 22 (46.8%) articular involvement, 20 (42.6%) renal involvement and 12 (25.5%) gastrointestinal tract involvement. The frequency of bulla and/or necrosis in the skin lesions was significantly high in patients with gastrointestinal and renal involvement (p<0.05). The risk of renal involvement was significantly high in patients whose DIF examination revealed IgM deposition (p<0.05). No association was found between systemic involvement and IgG, complement 3 (C3) and fibrinogen deposition.

Conclusion

Patients with HSP, having bullous and/or necrotic lesions and whose skin DIF investigations reveal IgM deposition along with IgA, should be surveyed for the risk of renal involvement.

     

Surgical treatment of CHILD nevus

We report a young girl with an unusual manifestation of CHILD syndrome in whom skin lesions showed involvement of the right side of her neck as well as symmetrically distributed ptychotropic involvement of the large body folds. Excision resulted in improvement and finally healing of skin lesions within the submammary folds, where breast reduction was also performed, whereas excision of axillary lesions and subsequent grafting with split skin turned out to be unsuccessful.     

Cutaneous sarcoid with varied morphology associated with hypercalcaemia and renal impairment

Sarcoidosis is a multisystem disorder of unknown aetiology, which presents with hilar lymphadenopathy, pulmonary infiltration, and ocular and cutaneous involvement. Cutaneous lesions often present as erythema nodosum, maculopapular, plaque, scar, subcutaneous nodule or lupus pernio. Most patients with cutaneous involvement have a single type of skin lesion, but some cases may have ≥ 2 types. We report a case of sarcoidosis presenting with various types of skin lesions. The case was also complicated by hypercalcaemia and renal dysfunction, and was successfully treated with oral corticosteroids. Presentation of various skin lesions may indicate systemic organ involvement requiring treatment with systemic corticosteroid.     

A retrospective randomly selected cohort study of D-penicillamine treatment in rapidly progressive diffuse cutaneous systemic sclerosis of recent onset

BACKGROUND: Several uncontrolled studies in systemic sclerosis have shown that D-penicillamine may cause improvement in skin sclerosis, decrease the rate of new visceral organ involvement, and improve overall survival. OBJECTIVES: To undertake a single-centre retrospective randomly selected cohort study to examine the effects of D-penicillamine treatment on skin and visceral organ involvement in patients with rapidly progressive systemic sclerosis of recent onset. METHODS: Eighty-four patients with diffuse cutaneous systemic sclerosis who had received D-penicillamine within 24 months of clinically detectable onset of skin sclerosis were randomly selected from the systemic sclerosis cohort followed at the Scleroderma Center of Thomas Jefferson University. Employing a previously described severity scale, disease severity and skin involvement were compared from initiation of D-penicillamine to end of study and a correlated matched t-test was used to establish statistical significance. RESULTS: At a mean+/-SD duration of D-penicillamine therapy of 29.2+/-5.5 months and at a median dose of 750 mg per day statistically significant improvement in skin (P<0.01) and cardiac, pulmonary and renal involvement (P<0.05) was observed. At last follow-up, 17 (20%) patients were still receiving D-penicillamine, 25 (30%) had discontinued it owing to disease improvement, and 18 (21%) had discontinued it owing to side-effects. CONCLUSIONS: In a population of patients with diffuse cutaneous systemic sclerosis, with progressive disease of recent onset, D-penicillamine treatment at a median dose of 750 mg per day caused a statistically significant reduction in skin involvement and improvement of renal, cardiac and pulmonary involvement.     

In vivo study of skin mechanical properties in patients with systemic sclerosis

BACKGROUND: Measurements of skin elasticity are more sensitive than the skin severity score and appropriate for evaluation of sclerodermatous skin. OBJECTIVE: Our purpose was to investigate the mechanical properties of the skin in patients with systemic sclerosis depending on the stage of the disease. METHODS: Seventeen patients, 8 with edematous phase and 9 with indurative phase of skin involvement, and 16 healthy subjects were studied. Clinical scoring of skin thickness and measurements of skin elasticity with a noninvasive suction device (Cutometer) were performed over 11 anatomic regions. RESULTS: Edematous phase was characterized by significantly lower immediate distention (Ue) and final distention (Uf), and higher viscoelastic to elastic ratio (Uv/Ue) of the skin compared with indurative phase, except for the forehead (8 mm probe). The changes in skin mechanical parameters for fingers were identical in both phases. Low values of skin distensibility correlated with severe skin thickness or hidebound skin. Results were influenced by body site and by the diameter of measuring probe used. CONCLUSION: The noninvasive method applied is suitable for objective and quantitative monitoring of skin involvement in patients with systemic sclerosis.     

Vitiligo as a systemic disease

Vitiligo is an acquired depigmentary skin disorder of unknown etiology. Vitiligo is not only a disease of melanocytes of the skin. Human melanocytes are derived from the neural crest and are located on various parts of the body. The involvement of skin melanocytes is the most visible one, but a systemic involvement of melanocytes can be observed. Some types of vitiligo (nonsegmental vitiligo) may also be associated with various diseases, mainly with autoimmune pathogenesis. Vitiligo represents a spectrum of many different disorders with different etiologies and pathogeneses, causing a common phenotype: the loss of melanocytes and/or their products. This phenotype is always consistent with a systemic involvement.     

Bullous amyloidosis

Amyloidosis may present with involvement of a variety of organ systems. Cutaneous involvement is a relatively common finding in patients with systemic amyloidosis. The occurrence of bullous skin lesions, however, is rare; only a few such cases have been previously reported. We describe a patient who presented with a subepidermal bullous skin disease initially thought to be bullous pemphigoid based on both clinical and histologic appearances. The patient subsequently developed the nephrotic syndrome. Biopsy specimens of the skin and kidney showed involvement of both organs with amyloid, and amyloid was later found in the spleen, heart, and nervous system. No subsequent evidence of myeloma was found in this patient. The clinical, histopathologic, immunofluorescent, and electron microscopic findings of systemic amyloidosis are discussed.     

Sea-blue histiocyte syndrome with cutaneous involvement. Case report with ultrastructural findings

A patient with infiltration of the skin resulting in eyelid swelling and facial nodules was recognized as a case of sea-blue histiocyte syndrome with cutaneous involvement. Typical sea-blue histiocytes were found in the skin and confirmation was provided by electron microscopy. Hepatosplenomegaly, lung infiltrates and bone marrow involvement were the other symptoms. The relationship between sea-blue histiocyte syndrome and adult Niemann-Pick disease is also discussed.     

Analysis of prognostic markers

One of several objectives of this study was to search for prognostic indicators for the various complications of PXE, particularly the retinal and cardiovascular aspects. The first question was whether the most visible sign of PXE—the skin lesion—was in any quantitative sense correlated with eye and vascular involvement. A positive correlation would be helpful in counseling patients who have extensive skin involvement that they would also be at greater risk for systemic complications. Or, if the data lacked correlation, it would suggest that the skin, eye, and vascular aspects proceed at completely independent rates and that the extent of involvement in one area has no prognostic relationship to any other area.     

Study of desmoglein 1 and 3 antibody levels in relation to disease severity in Indian patients with pemphigus

OBJECTIVES: To conduct a cross-sectional study to compare Dsg1 and Dsg3 antibody levels independently with severity of disease activity in pemphigus vulgaris (PV) and pemphigus foliaceus (PF). METHODS: Blood samples from 44 patients with pemphigus (PV-38, PF-6) were analyzed using ELISA. The severity of skin and mucosal disease was graded using a score from 0 to 3. RESULTS: A statistically significant correlation between increase in Dsg 3 antibody titres with severity of oral involvement and Dsg 1 titres with severity of skin involvement was found in both PV and PF patients (p < 0.01). However, we were unable to demonstrate a relationship between increased titres of Dsg1 and Dsg 3 antibodies with oral and skin involvement respectively. CONCLUSION: This study suggests that the severity of skin and oral disease in pemphigus is determined by the quantities of Dsg1 and Dsg3 antibodies respectively.     

Cutaneous sinus histiocytosis and chronic uveitis

Sinus histiocytosis with massive lymphadenitis or Rosai-Dorfman disease (RDD) is a rare, benign, proliferative histiocytic disease of unknown origin. It predominately affects the lymph nodes The skin is the extranodal organ most frequently involved, although a few patients present with only lesions of the skin and soft tissues, without involvement of the lymph nodes (purely cutaneous RDD). Ocular involvement is rare, and generally localized to the eyelid. We report an unusual case of cutaneous sinus histiocytosis without nodal involvement and with bilateral uveitis in a girl. As far as we know, this is the first case reported in which cutaneous and ocular RDD occurred without nodal involvement.     

Systemic Juvenile Xanthogranuloma with Fatal Outcome

Abstract: Juvenile xanthogranuloma is a benign and self‐limited disease which usually appears in the skin of children. Visceral involvement has been rarely reported, as has fatal outcome in some affected individuals. We report a case of systemic juvenile xanthogranuloma in a female newborn with mainly skin, bone marrow, and liver involvement, leading to death at the age of 2 months.