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Case 1, (27th Annual Uniformed Services Dermatology Seminar, 2003) FAMILIAL BENIGN CHRONIC PEMPHIGUS (HAILEY-HAILEY DISEASE)

Twede JVT - MedPix (2006)

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: Case 1, (27th Annual Uniformed Services Dermatology Seminar, 2003) FAMILIAL BENIGN CHRONIC PEMPHIGUS (HAILEY-HAILEY DISEASE)

History: 19 year-old Caucasian female with a five year history of relapsing courses of pruritic crops of small papules and vesicles which rupture and crust, over a period of several weeks. At the onset of the disease, the lesions were noted on the back and over the past few years have become more widespread to include the neck, sub-mammary chest, and abdomen. Exacerbations have been associated with heat (warmer months and sports seasons), as well as with friction (necklace wear and tight fitting pants).

Findings: Histopath: The biopsy shows multiple foci of epidermal acanthosis and areas of partial acantholysis with intracellular edema. Suprabasilar clefts with acantholytic cells singly and in clumps line the lacunae. A broad vesicle with clumps of acantholytic cells floating free within the vesicle is also noted. There is no adenxal involvement. There is a mild superficial perivascular lymphocytic infiltrate in the dermis.

Ddx: • pemphigus vulgaris • transient acantholytic dyskeratosis • spongiotic dermatitis

Dxhow: biopsy proven, peer-reviewed

Exam: Examination: Scattered crops of erythematous papules with crusting coalescing into plaques without evidence of drainage or foul odor; mild hyperpigmentation at prior crops of lesions; no lymphadenopathy, nail banding, hair, facial, or oral involvement.

No MeSH data available.


Histopath: The biopsy shows multiple foci of epidermal acanthosis and areas of partial acantholysis with intracellular edema. Suprabasilar clefts with acantholytic cells singly and in clumps line the lacunae. A broad vesicle with clumps of acantholytic cells floating free within the vesicle is also noted. There is no adenxal involvement. There is a mild superficial perivascular lymphocytic infiltrate in the dermis.
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MPX2415_synpic23416: Histopath: The biopsy shows multiple foci of epidermal acanthosis and areas of partial acantholysis with intracellular edema. Suprabasilar clefts with acantholytic cells singly and in clumps line the lacunae. A broad vesicle with clumps of acantholytic cells floating free within the vesicle is also noted. There is no adenxal involvement. There is a mild superficial perivascular lymphocytic infiltrate in the dermis.


Case 1, (27th Annual Uniformed Services Dermatology Seminar, 2003) FAMILIAL BENIGN CHRONIC PEMPHIGUS (HAILEY-HAILEY DISEASE)

Twede JVT - MedPix (2006)

Histopath: The biopsy shows multiple foci of epidermal acanthosis and areas of partial acantholysis with intracellular edema. Suprabasilar clefts with acantholytic cells singly and in clumps line the lacunae. A broad vesicle with clumps of acantholytic cells floating free within the vesicle is also noted. There is no adenxal involvement. There is a mild superficial perivascular lymphocytic infiltrate in the dermis.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=MPX2415&req=5

MPX2415_synpic23416: Histopath: The biopsy shows multiple foci of epidermal acanthosis and areas of partial acantholysis with intracellular edema. Suprabasilar clefts with acantholytic cells singly and in clumps line the lacunae. A broad vesicle with clumps of acantholytic cells floating free within the vesicle is also noted. There is no adenxal involvement. There is a mild superficial perivascular lymphocytic infiltrate in the dermis.

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: Case 1, (27th Annual Uniformed Services Dermatology Seminar, 2003) FAMILIAL BENIGN CHRONIC PEMPHIGUS (HAILEY-HAILEY DISEASE)

History: 19 year-old Caucasian female with a five year history of relapsing courses of pruritic crops of small papules and vesicles which rupture and crust, over a period of several weeks. At the onset of the disease, the lesions were noted on the back and over the past few years have become more widespread to include the neck, sub-mammary chest, and abdomen. Exacerbations have been associated with heat (warmer months and sports seasons), as well as with friction (necklace wear and tight fitting pants).

Findings: Histopath: The biopsy shows multiple foci of epidermal acanthosis and areas of partial acantholysis with intracellular edema. Suprabasilar clefts with acantholytic cells singly and in clumps line the lacunae. A broad vesicle with clumps of acantholytic cells floating free within the vesicle is also noted. There is no adenxal involvement. There is a mild superficial perivascular lymphocytic infiltrate in the dermis.

Ddx: • pemphigus vulgaris • transient acantholytic dyskeratosis • spongiotic dermatitis

Dxhow: biopsy proven, peer-reviewed

Exam: Examination: Scattered crops of erythematous papules with crusting coalescing into plaques without evidence of drainage or foul odor; mild hyperpigmentation at prior crops of lesions; no lymphadenopathy, nail banding, hair, facial, or oral involvement.

No MeSH data available.