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 29, Issue 5, 2023
Anti-laminin 332 antibodies in graft-versus-host disease-associated bullous pemphigoid after allogeneic peripheral blood stem cell transplantation
We report a 48-year-old woman with bullous pemphigoid (BP) with antibodies against the B3 subunit of laminin 332 after the development of graft-versus-host disease (GVHD). She was diagnosed with recurrent acute lymphoblastic leukemia at 40 years of age and underwent two rounds of allogeneic peripheral blood stem cell transplantations (PBST). Two and a half years after the second PBST, multiple tense blisters appeared on her face, hands, and lower legs. The diagnosis of BP was based on hematoxylin eosin and immunofluorescence staining and immunoblotting analyses. A combination regimen of topical corticosteroids (clobetasol propionate) and tetracycline/niacinamide was administered. Complete clinical resolution was achieved after four weeks of therapy without the use of immunosuppressive drugs. To maintain the graft-versus-tumor effect, topical corticosteroids and immunomodulatory drugs are preferred for BP after hematopoietic stem cell transplantation considering the risk of recurrence of hematologic malignancies. To date, there have been no reports of successful treatment of GVHD-associated BP without immunosuppressive drugs. Chronic GVHD is characterized by the production of autoantibodies. Furthermore, this autoimmune subepidermal blistering disease, BP, may be a manifestation of chronic GVHD. However, the precise mechanism of autoantibody production in chronic GVHD is not yet fully elucidated.
Cutaneous lesions of secondary syphilis are highly infectious and can mimic many skin disorders, making the diagnosis more difficult. They typically present as generalized, nonpruritic erythematous-to-copper-colored macules and papules, characteristically involving palms and soles. In 80% of patients the rash develops insidiously. However, rare forms of secondary syphilis present as rapidly progressive papulopustular lesions. These forms of syphilis are usually associated with human immunodeficiency virus infection and immunosuppression. We report a case of secondary syphilis presenting with an acute, rapidly progressive purpuric eruption mimicking leukocytoclastic vasculitis. A 61-year-old man presented with a 6-day history of nonpruritic rash on his chest and lower extremities associated with fatigue, sore throat, and night sweats. Examination revealed purpuric papules, extending from the dorsal feet to the hips; mucosal surfaces were not involved. A diagnosis of cutaneous small-vessel vasculitis was favored with possible triggers of IgA vasculitis. Initial work-up showed acute kidney injury and microscopic hematuria. Renal biopsy showed IgA nephropathy with mesangioproliferative glomerulonephritis. The patient's rash progressed to cover almost his entire body sparing palms and soles. Skin biopsy showed heavy perivascular lymphoplasmacytic infiltrate, capillary endothelial cell swelling, and sparse perivascular neutrophilic nuclear dust. Spirochetal stain highlighted scattered epidermal and dermal organisms.
Two congenital cases of pigmented epithelioid melanocytoma with unique clinical and genetic features
Pigmented epithelioid melanocytomas (PEM) are intermediate-grade melanocytic lesions with frequent lymph node involvement and rare metastases that tend to follow an indolent course with a favorable outcome. We report two unique cases of congenital PEM with PRKCA fusion transcripts: a multifocal PEM with an aggressive incompletely resectable scalp tumor and a solitary palmar PEM with newly reported ITGB5-PRKCA fusion. Through these case reports and a summary of previously reported cases, we outline the spectrum of disease of PEM and highlight the key clinical and histopathologic features associated with PEM with PRKCA fusion transcripts. We also discuss the treatment options and suggest that surgical excision without further adjuvant systemic treatment is reasonable first-line therapy given the favorable prognosis.
Generalized perforating granuloma annulare associated with latent tuberculosis successfully treated with isoniazid: case report and review
Generalized perforating granuloma annulare (GPGA) is a very rare form of granuloma annulare, with only 31 reported cases to the best of our knowledge. Furthermore, GPGA is a chronic disease that mimics many diseases, with no known exact etiology, resulting in a lack of specific clinical criteria leading to a lack of guidelines for diagnosis and therapy. In GPGA, papules are the predominant lesions followed by central crusting/scaling or umbilication; pustules, plaques, annular lesions or nodules are less frequent. We report a 66-year-old woman who presented with a 7-month history of mostly asymptomatic generalized infiltrated, flesh-colored to red-brown umbilicated or crusted papules. Histopathological findings were compatible with perforating granuloma annulare. Diagnostic workup revealed latent tuberculosis. To the best of our knowledge, this is the second published case of GPGA associated with latent tuberculosis and the first one that was successfully treated by isoniazid monotherapy. From our case we can speculate and support the theory that GPGA is a phenotypic granulomatous response to multiple etiologies and/or antigenic stimulation and that testing for tuberculosis should be seriously considered in the evaluation of patients with GPGA.
Darier disease is an autosomal dominant blistering disorder linked to mutation of the endoplasmic reticulum calcium pump, SERCA2, which compromises keratinocyte adhesion and differentiation. Beyond the typical keratotic and eroded skin lesions, patients with Darier disease often present with psychiatric co-morbidities. Herein, we present a biopsy-confirmed case of Darier disease in a patient with bipolar disorder, whose cutaneous disease dramatically worsened upon initiation of lithium therapy. In consultation with the patient's psychiatrist, lithium was tapered, leading to rapid improvement in her skin. This case highlights the potential for lithium to complicate management of Darier disease and underscores the need for dermatologists to collaborate with psychiatrists to optimize both cutaneous and mental health in patients.
A case of monkeypox and cytomegalovirus coinfection manifesting with crusted lesions mimicking rupiod syphilis
Human monkeypox is a viral zoonosis that has recently emerged worldwide. Clinical cutaneous features include papules, vesicles, and pustules. However, atypical manifestations mimicking other infectious diseases are being reported more frequently. We present a 41-year-old man patient with untreated HIV with generalized rupioid crusted ulcerated plaques with perineal ulceration that were found to represent monkeypox and cytomegalovirus infections.
Hydroxychloroquine (HCQ) is an antimalarial agent that is commonly used in the management of rheumatic skin disease. Few reports exist documenting exacerbation of dermatomyositis (DM) related to HCQ. Herein, we describe three adult patients with worsening DM cutaneous disease after starting HCQ and resolution or improvement with cessation. The time to exacerbation ranged from two weeks to nine months after the initiation of HCQ 400mg/day. Two of the three patients had antibodies to transcription intermediary factor 1γ (TIF1γ) and the other had antibodies to anti-nuclear matrix protein 2 (NXP2). After discontinuation of HCQ, the time to improvement or resolution of cutaneous symptoms ranged from six weeks to six months. Hydroxychloroquine may be associated with worsening cutaneous features in DM. In patients who are not improving despite escalation of immunosuppressive medications, or are worsening, we recommend a trial of discontinuing HCQ.
Erythema elevatum diutinum (EED) is a rare cutaneous neutrophilic vasculitis with many associated diseases reported in the literature. We report a 65-year-old woman with painful and itchy lesions on her elbows, hands, knees, and foot for a year. Histopathologic examination confirmed the diagnosis of erythema elevatum diutinum and treatment with dapsone produced significant clinical improvement within few weeks. Erythema elevatum diutinum is a rare disease that should be considered in patients with violaceous nodular plaques located over the extensor regions of the limbs. Knowledge of this unusual pathology and its association helps to avoid misdiagnosis and late treatment.
Cervical cancer remains one of the most common malignancies diagnosed in women as well as a leading cause of cancer related deaths in women worldwide. Cutaneous metastasis associated with cervical malignancy is a remarkably rare phenomenon. We present a patient whose cutaneous signs led to the diagnosis of metastatic adenocarcinoma of the cervix.
Pigmented purpuric lichenoid dermatitis (PPLD) is a rare subtype of pigmented purpuric dermatosis, which classically presents with a mixture of lichenoid papules and patches on the bilateral lower extremities. Herein, we describe an unusual case of a 47-year-old man with PPLD who presented with 1-3mm discrete papules without the presence of larger patches. The diagnosis of PPLD should be considered for patients presenting with bilateral symmetric discrete papules on the legs.
Pemphigus foliaceus is a superficial blistering disorder characterized by erosions and scaling in a seborrheic distribution. The condition typically occurs in healthy individuals but issues arise from delayed diagnosis. Many cases remain undiagnosed or misdiagnosed due to the lack of awareness of the condition. With use of common diagnostic tools, pemphigus foliaceus can be easily identified and monitored. Histological analysis exhibits "chicken wire" patterning along keratinocytes in the upper epidermis, whereas immunofluorescence study displays subcorneal acantholysis. Pemphigus foliaceus is confirmed via ELISA studies revealing the presence of autoantibodies against desmoglein 1. Once correctly diagnosed, typically the condition is responsive to corticosteroid therapy. However in recalcitrant cases such as in ours, adjunctive immunosuppressive therapy with dapsone or rituximab may be indicated.
Cutaneous manifestations of syphilis are varied and may present with non-specific features. We describe a 45-year-old man who presented with erythematous scaly plaques and nodules on his scalp. In previously reported cases, there were only descriptions of nodules as well as tumors. However, in our case, the patient presented with plaques and nodules on his scalp that quickly resolved with treatment for syphilis. It is important to recognize and treat syphilis at an early stage.
Ficus carica L photodermatitis: a report of five cases with histopathologic study and review of the literature.
Phytophotodermatitis is a condition caused by contamination of the skin with phototoxic plant substances, followed by exposure to ultraviolet rays. Ficus carica L 1753, belonging to the Moraceae family, can be responsible for acute photodermatitis. We present five cases of photodermatitis caused by contact with Ficus carica L and subsequent exposure to sunlight. A histopathologic study and review of the literature are included.
Livedoid vasculopathy is a painful thrombo-occlusive vascular disorder characterized by spontaneous thrombosis in medium-size arterioles, which causes localized hypoxia and skin ulceration. As livedoid vasculopathy is rare, case reports are the primary means of expanding collective knowledge about its presentation and response to various therapies.