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This is an overview of the cutaneous porphyrias. It is a narrative review based on the published literature and my personal experience; it is not based on a formal systematic search of the literature. The cutaneous porphyrias are a diverse group of conditions due to inherited or acquired enzyme defects in the porphyrin–haem biosynthetic pathway. All the cutaneous porphyrias can have (either as a consequence of the porphyria or as part of the cause of the porphyria) involvement of other organs as well as the skin. The single commonest cutaneous porphyria in most parts of the world is acquired porphyria cutanea tarda, which is usually due to chronic liver disease and liver iron overload. The next most common cutaneous porphyria, erythropoietic protoporphyria, is an inherited disorder in which the accumulation of bile-excreted protoporphyrin can cause gallstones and, rarely, liver disease. Some of the porphyrias that cause blistering (usually bullae) and fragility (clinically and histologically identical to porphyria cutanea tarda) can also be associated with acute neurovisceral porphyria attacks, particularly variegate porphyria and hereditary coproporphyria. Management of porphyria cutanea tarda mainly consists of visible-light photoprotection measures while awaiting the effects of treating the underlying liver disease (if possible) and treatments to reduce serum iron and porphyrin levels. In erythropoietic protoporphyria, the underlying cause can be resolved only with a bone marrow transplant (which is rarely justifiable in this condition), so management consists particularly of visible-light photoprotection and, in some countries, narrowband ultraviolet B phototherapy. Afamelanotide is a promising and newly available treatment for erythropoietic protoporphyria and has been approved in Europe since 2014.

Cutaneous leishmaniasis (CL) is an infectious disease caused by various species of leishmania protozoan parasites. Lupoid CL is a rare form of CL that has a stunning similarity to other granulomatous cutaneous conditions of infectious or inflammatory origin. Verruca plana, also known as a “flat wart”, is a benign proliferation of the skin resulting from infection with human papilloma virus (HPV). Herein, we presented a case of lupoid CL mimicking verruca plana on the face.

How the main components in systemic sclerosis—namely autoimmunity, vasculopathy, and fibrosis—fit together is still not sufficiently clear. However, vascular treatment options are well established, the body of evidence for the efficacy of immunomodulatory approaches is increasing, and now at least one hopeful substance that may directly interfere with fibrosis is being tested. Although we still wait for important breakthroughs, there is grounds for hope that better therapeutic options will be available in the near future.

Cutaneous leishmaniasis (CL) is an infectious disease caused by various species of leishmania protozoan parasites. Lupoid CL is a rare form of CL that has a stunning similarity to other granulomatous cutaneous conditions of infectious or inflammatory origin. Verruca plana, also known as a “flat wart”, is a benign proliferation of the skin resulting from infection with human papilloma virus (HPV). Herein, we presented a case of lupoid CL mimicking verruca plana on the face.

Clonal mast cell activation syndromes and indolent systemic mastocytosis without skin involvement are two emerging entities that sometimes might be clinically difficult to distinguish, and they involve a great challenge for the physician from both a diagnostic and a therapeutic point of view. Furthermore, final diagnosis of both entities requires a bone marrow study; it is recommended that this be done in reference centers. In this article, we address the current consensus and guidelines for the suspicion, diagnosis, classification, treatment, and management of these two entities.

Cutaneous T cell lymphomas (CTCLs) are a heterogeneous group of extranodal non-Hodgkin’s lymphomas that are characterized by a cutaneous infiltration of malignant monoclonal T lymphocytes. They typically afflict adults with a median age of 55 to 60 years, and the annual incidence is about 0.5 per 100,000. Mycosis fungoides, Sézary syndrome, and primary cutaneous peripheral T cell lymphomas not otherwise specified are the most important subtypes of CTCL. CTCL is a complicated concept in terms of etiopathogenesis, diagnosis, therapy, and prognosis. Herein, we summarize advances which have been achieved in these fields.

Introduction: Reactive angioendotheliomatosis (RAE) is a benign vascular disorder with a varied clinical presentation that has been associated with a wide range of systemic illnesses. It is characterized histologically by intravascular and extravascular hyperplasia of endothelial cells and pericytes, and this entity only affects the skin. We present the rare case of a woman with both RAE and panniculitis associated with anticardiolipin antibodies in the setting of antiphospholipid syndrome (APLS). Case description: A female in her forties presented for a second opinion of lumps in her skin. The first lump was noted on her right thigh four years prior to presentation, and biopsy demonstrated longstanding septal and lobular panniculitis. Studies that followed this finding revealed a diagnosis of APLS, and the patient had been placed on 400 mg per day of hydroxychloroquine and a daily low-dose of aspirin. She presented with a new subcutaneous plaque on the left lower back, and biopsy of this lesion was suggestive of RAE. She had no history of significant thrombotic events and had never been treated with an anticoagulant. Work-up was negative for other connective tissue diseases that are known for producing subcutaneous nodular lesions. Discussion: RAE is very rare, with less than 50 cases reported in total; five of these in association with APLS. In the context of APLS, RAE is likely a result of the procoagulant state induced by the presence of antiphospholipid antibodies. This case of both RAE and panniculitis associated with anticardiolipin antibodies in the setting of APLS describes rare manifestations of APLS as isolated incidents in a relatively healthy patient.